Parmakların fibro-osseöz psödotümörü

Parmakların fibro-osseöz psödotümörü özellikle iskelet dışı osteosarkom ile karışabilen ve ayırıcı tanıda ciddi sorunlara ve çelişkilere neden olabilen nadir bir benign lezyondur. Sol el işaret parmağında ağrılı şişme ile kliniğimize başvuran 57 yaşında erkek hastanın direkt radyografisinde sol el işaret parmağı proksimal falanks palmar bölgede şüpheli kalsifiye alanlar içeren yumuşak doku şişliği görüldü. Cerrahi olarak çıkartılan 2x1.5x0.7 cm boyutlarındaki biyopsi örneğinin gri beyaz renkte olduğu ve nispeten düzenli dış yüzeye sahip olduğu izlendi. Mikroskopik olarak mezenkimal nitelikler izlenen lezyon kısmen dejenere, kısmen kalsifiye/ ossifiye miksoid stromaya sahipti. Hafif-orta derecede nükleer atipi ve nispeten sık mitoz bulunan hücresel fibroblastik alanlar ve bu alanların arasında düzensiz gruplar oluşturan osteoklast benzeri dev hücreler izlendi. Histopatolojik, radyografik ve klinik bulgular ışığında son tanı olarak olguya parmakların fibro-osseöz psödotümörü tanısı konuldu.Fibro-osseous pseudotumor of the digits is a conflicting and rare benign lesion leading to serious problems and conflicts in differential diagnosis, especially with extraskeletal osteosarcoma. A 57-year-old man presented with a painful swelling in the proximal phalange of his left index finger. Direct radiography taken on admission revealed a soft tissue swelling with suspicious calcification in the proximal phalange palmar aspect of the index finger of left hand The excised specimen of, 2x1.5x0.7 cm was gray-white with a rough, somewhat irregular outer surface. Microscopic examination revealed a mesenchimal tumorous lesion with a partially degenerated and partially calcified/ ossified myxoid stroma. There were cellular areas composed of fibroblastic spindle cells, with mild-moderate nuclear atypia and moderate mitotic activity. Those cellular areas also contained osteoclast like multinucleated giant cells forming disordered groups. The final diagnosis based on histopathologic, radiographic and clinical findings was fibro-osseous pseudotumor of the digits

Smith-Lemli-Opitz syndrome: A case report

Smith-Lemli-Opitz sendromu otozomal resesif geçen, çok sayıda doğuştan malformasyonun eşlik ettiği nadir görülen bir sendromdur. Smith-Lemli-Opitz sendromlu olgularda kolesterol biyosentezinin son basamağında 7-dehidrokolesterolü kolesterole çeviren ve geni 11q13'de lokalize bir enzim olan 7-dehidrokolesterol redüktazın doğuştan eksikliği mevcuttur. Otuz beş haftalık 1500 gr doğan bebeğin cildi kuru ve parşömen kâğıdı görünümünde, mikrognati, hipertelörizm, düşük kulak, antevert burun delikleri, belirgin filtrum, yarık damak, el parmaklarında ulnar deviasyon ve distal fleksiyon kontraktürü, çomaklaşma, ayaklarda pes ekino varus deformitesi ve çekiç topuk, sağ ayakta sindaktili, ambigius genitalya mevcuttu. İncelemelerde total kolesterol 108 mg/dl LDL kolesterol 48.8 mg/dl bulundu. Yirmi beşinci gün hayatını kaybeden olgunun otopsisinde iki loblu sağ akciğer, atriyal septal defekt, patent duktus arteriyozus, üreterlerde iki taraflı yerleşim anomalisi, sol sürrenalde insitu saptandı.Smith-Lemli-Opitz syndrome is a rare autosomal recessive disorder which is accompanied by many congenital malformations. The syndrome is caused by a congenital deficiency of 7-dehydrocholesterol reductase which is localized on 11q13 gene and it is the final enzyme in the sterol synthetic pathway that converts 7-dehydrocholesterol to cholesterol. The infant was born on the 35. week of gestation and weighed 1500 grams. Anomalies evident on physical examination included dry and thinned skin, micrognathia, hypertelorism, low-set ears, anteverted nares, a long philtrum, cleft palate, ulnar deviation and distal flexion contracture of the fingers, equinovarus deformity and hammer toe, syndactyly of the right toes and ambiguous genitalia. Total cholesterol concentration was 108 mg/dl and LDL-cholesterol was 48.8 mg/dl. The infant died on postnatal day 25 and her autopsy revealed a right lung with two lobes, atrial septal defect, patent ductus arteriosus, bilateral dislocation of the ureter and insitu in the left adrenal gland

Cytological Diagnosis of Small Cell Carcinoma of Urinary Bladder in a Patient with CLL

Small cell carcinoma of the urinary bladder (SCCUB) is an extremely rare bladder malignancy characterized by an aggressive clinical behavior. So, it is important to diagnose this high grade disease by urinary cytology. We report a case of SCCUB in an old man with chronic lymphocytic leukemia (CLL) in remission, while bladder tumor was diagnosed by cytology. With this article, we aimed to review and to update the literature concerning this tumor

A case of breast carcinoma presenting as carcinoma en cuirasse

Carcinoma en cuirasse is a rare and unique form of cutaneous metastases, which surrounds the trunk in a firm and leathery fibrotic fashion, like an armor. We report a case of carcinoma en cuirasse in a 53-years-old female patient who was admitted to Dermatology outpatient clinic with pruritic, red, firm papules and plaques on the left breast and arm, extending to upper abdomen and medial part of right breast. At time of admission the patient did not have a history of an internal malignancy. Histopathological diagnosis of the incisional biopsy material from the plaque lesion was consistent with invasive ductal carcinoma. Imaging studies showed axillary, paraaortic, aortocaval lymph node involvement, pleural effusion, lung and liver metastases. The patient was referred to Medical Oncology Department and was treated with cyclophosphamide and adriamycine chemotherapy. This case of carcinoma en cuirasse preceeding the diagnosis of metastatic breast carcinoma and leading to diagnosis of malignancy is rare and important. Prognosis depends on the type of primary tumor and it’s biological behavior, predictably, poor prognosis is expected in this case of metastatic invasive ductal carcinoma

The expression of GST isoenzymes in acinar adenocarcinoma, intraepithelial neoplasia, and benign prostate tissue: Correlation of clinical parameters with GST isoenzymes

This study investigated the immunohistochemical staining characteristics of glutathione-S-transferase (GST) alpha, pi, mu, and theta in prostatic acinar adenocarcinoma (PCA), prostatic intraepithelial neoplasia (PIN), and benign prostatic tissues from 19 patients. Relationships between GST isoenzyme expression in benign, PIN, and PCA tissue were examined by the Wilcoxon signed-rank test and clinicopathological data were examined by the Spearman correlation rank test. When the benign, PIN, and PCA tissues from these cases were compared according to their staining intensity, GST alpha, pi, mu, and theta expressions in tumor cells were significantly lower than in benign epithelial cells (P 0.05). The GST alpha class displayed the lowest level of expression in PIN and PCA. Expression of GST pi was lower in PCA tissue than in PIN and benign epithelial tissue (P 0.05). We hypothesize that carcinogenesis in the prostate results from impaired cellular handling of mutagenic agents owing to reduction or loss of expression of multiple GST isoenzymes and other detoxifying and antimutagenesis agents. This study confirms the down-regulation of GST isoenzymes in PCA of the prostate and shows that the loss of GST isoenzyme expression is a phenotype associated with malignant transformation. There was no statistical relationship between GST isoenzyme expression and the clinicopathological data (age, Gleason score, and total serum prostate-specific antigen levels) (P > 0.05). © TÜBİTAK

A generalized case of purpura annularis telangiectoides of Majocchi

Purpura annularis telangiectoides is a rare form of pigmented purpuric dermatoses which usually presents as annular patches with central clearing on the lower extremities. We present an atypical presentation of purpura annularis telangiectoides with generalized lesions in an elderly woma