Izraženost receptora estrogena i progesterona u ljudskom inače nespecificiranom duktalnom invazivnom karcinomu dojke: postoji li razlika između žena u predmenopauzi i postmenopauzi ?

Determination of hormone receptors is of utmost importance in planning therapy in patients with breast cancer. The aim of the study was to assess the expression of estrogen (ER) and progesterone (PR) receptors in ductal invasive breast carcinoma not otherwise specified (NOS) according to patient menopausal state and tumor histopathology. The study included 549 patients treated at University Department of Surgery, Rijeka University Hospital Center, between January 1, 2000 and January 1, 2005. The patients were diagnosed with breast cancer and underwent mastectomy. ER and PR status was determined by immunohistochemistry. Study results showed no statistically significant differences in the expression of ER and PR, tumor size and grade of histologic differentiation between premenopausal and postmenopausal women. However, tumor size and grade of histologic differentiation differed significantly according to the expression of hormone receptors. Tumors greater than 5 cm in size were mostly ER- in premenopausal (P=0.012) and PRin postmenopausal (P=0.044) patients. Poorly differentiated cancers were associated with ER-PRstatus in both premenopausal and postmenopausal patients (P<0.001). Hormone dependent tumors (ER+PR+) were of smaller diameter and lower histologic grade, while hormone independent tumors (ER-PR-) had greater diameter and higher histologic grade, the difference being statistically significant (P=0.004 and P<0.001, respectively). Study results on the characteristics of ductal invasive carcinoma according to hormone status were consistent with those described in the literature. Considering controversies about the role of steroid receptors in endocrine therapy response, our future objective is assessment of the 5-year prognosis in our patients.Određivanje hormonskih receptora iznimno je važno u planiranju liječenja kod bolesnica s rakom dojke. Cilj ove studije bio je procijeniti izraženost receptora estrogena (ER) i progesterona (PR) u inače nespecificiranom duktalnom invazivnom karcinomu dojke (NOS) u odnosu na menopauzalni status žene i histopatologiju tumora. U studiju je bilo uključeno 549 žena liječenih na Klinici za kirurgiju Kliničkog bolničkog centra Rijeka između 1. siječnja 2000. i 1. siječnja 2005. godine. Kod bolesnica je bio dijagnosticiran rak dojke i one su podvrgnute mastektomiji. Status ER i PR određen je imunohistokemijskim metodama. Rezultati nisu pokazali nikakvih značajnih razlika u izraženosti ER i PR, veličini tumora i stupnju histološke diferencijacije između žena u predmenopauzi i onih u postmenopauzi. Međutim, veličina tumora i stupanj histološke diferencijacije razlikovali su se značajno u odnosu na izraženost hormonskih receptora. Tumori veći od 5 cm uglavnom su bili ER- kod žena u predmenopauzi (P=0,012) i PR- kod onih u postmenopauzi (P=0,044). Slabo diferencirani karcinomi bili su udruženi sa statusom ER-PR- kod bolesnica u predmenopauzi kao i kod onih u postmenopauzi (P<0,001). Hormonski ovisni tumori (ER+PR+) bili su manjeg promjera i nižeg histološkog stupnja, dok su hormonski neovisni tumori (ER-PR-) imali veći promjer i viši histološki stupanj, i ta je razlika bila statistički značajna (P=0,004 odnosno P<0,001). Rezultati dobiveni za značajke duktalnog invazivnog karcinoma prema hormonskom statusu bili su sukladni onima u literaturi. S obzirom na proturječja o ulozi steroidnih receptora u endokrinom odgovoru na terapiju naš je budući cilj procijeniti 5-godišnju prognozu kod naših bolesnica

Pilomatrixoma: A Benign Appendageal Tumor not Uncommon in Children

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Pediatric Solid Pseudopapillary Neoplasm[Spn] of The Pancreas : Case Report and Literature Review

Background: Pediatric pancreatic malignancies are extremely rare with an age population-adjusted incidence of 0.018 cases per 100, 000. In adolescent girls, they usually present as solid pseudopapillary neoplasms[SPNs], low-grade malignant tumors of the exocrine pancreas. Due to their rarity and nonspecific clinical presentation, SPNs are frequently misdiagnosed. The treatment of choice is a complete surgical resection with 5-year survival rate of 97%. Case Report: We report a 17-year-old girl with SPN presenting with jaundice and itchy skin. She was initially misdiagnosed with hepatoblastoma, and treated with neoadjuvant chemotherapy. The girl later underwent surgical excision, and is in complete remission seven years after the diagnosis. Conclusion: Although rare, SPNs need to be included in the differential diagnosis of abdominal masses in children. MRI detected, well-defined, encapsulated, heterogeneous mass, especially in adolescent females, should always be suspicious for SPN. Further prospective multi-institutional studies are required in order to standardize preoperative evaluation and reduce the high misdiagnosis rate

Burns in children and their treatment

Opekline kože jedne su od vrlo čestih povreda kod djece svih uzrasta, a jedne od najčešćih kod manje djece. Ovisno o načinu nastanka, površini zahvaćenosti i duljini kontakta s toplinskim izvorom, opekline ponekad dovode do velikih metaboličkih promjena i pojačanog neuroendogenog odgovora organizma, pa govorimo o opeklinskoj bolesti. Prema dubini opečene površine opekline svrstavamo u opekline I., II., III. i IV. stupnja. Osim dubine, procjena opeklinske bolesti ovisi i o zahvaćenoj površini. Za procjenu veličine opečene površine koriste se razna pravila ili sheme, modificirane za djecu, jer osim dijelove tijela uzimaju u obzir I godine starosti. Tako je, uzimajući u obzir specifičnosti dječje dobi, težina opeklina kod djece podijeljena u tri kategorije: lake, umjerene i teške. Opekline I. i II. stupnja uglavnom se liječe konzervativno, za razliku od opeklina III. stupnja koje zahtijevaju operativno liječenje, u cilju uklanjanja nekrotičnog tkiva. No potreba da se ekscizija učini do u zdravo najčešće dovodi do povećanja površine koju treba pokriti. Posljedično tome dolazi i do nastanka većih ožiljaka, što je još uvijek predmet brojnih istraživanja kad je u pitanju njihovo liječenje. Važno je, dakle, izbjeći kirurški debridman kod djece, naročito ako znamo da postoji razlika u dječjoj fiziologiji i patofiziologiji opeklinskih rana. Upravo stoga, imajući u vidu da djeca nisu “odrasli u malom”, opekline zahtijevaju i drugačiji pristup liječenja, o čemu se govori u ovom preglednom članku.Skin burns are among the most common injuries in children of all ages, and one of the most common in younger children. Depending on the mode of occurrence, area of involvement and length of contact with the heat source, burns sometimes lead to major metabolic changes and increased neuroendogenous response of the body, which is referred to as the “burn disease”. Depending on the depth of tissue damage, burns are categorized into four grades: I, II, III and IV. Except for the depth, the area of involvement is also very important in the estimation of the burn disease. The size of burn involvement is measured by using various rules and schemes that are modified for children, taking into consideration not only the different parts of the body but also the age of the child. Altogether, burn wounds in children are clinically divided into three categories: mild, moderate and heavy. The grade I and II burns are mostly treated conservatively, whereas those of grade III need surgical treatment in order to remove the necrotic tissue. Surgical excision up to the normal tissue very often produces a larger area that should be replaced. As a consequence, the scars are larger and their treatment is still a subject of numerous research studies. It is very important to avoid surgical debridement in children, especially because the physiology and pathophysiology of the burns’ wound in children are different. Thus, burns in children have their own specificities and, considering that children are not “adults in a small”, require a different approach to treatment, which is discussed in this article

Additional Cytomorphological Criteria in Diagnosis of Pilomatricoma – Benign Tumor with Bad Reputation

Pilomatricomas (PM) are benign skin appendageal tumors, with differentiation towards hair-forming cells, usually found in children. They are frequently misdiagnosed by clinicians, and there are also many reports of false positive diagnoses made on fine needle aspiration (FNA) cytology. PM are often mistaken for »small round blue cell« tumors in children, or for Merkel cell carcinoma, basalioma and metastatic small cell carcinoma in adults, with possible over-aggressive therapeutic approach. We present 6 cases of PM, correctly diagnosed preoperatively by FNA. Clinical, cytomorphologic and basic morphometric features were analyzed, and compared with 4 cases of malignant tumors with similar clinical presentation. Morphometric data (longest nuclear diameter) did not prove to be helpful, while basophilic cytoplasmatic protrusions, observed in all 6 analyzed cases, could be useful additional cytomorphologic feature of PM. We concluded that cytomorphologic characteristics of PM are reliable enough for correct preoperative diagnosis in adequate specimens, however the best results are achieved when FNA is performed by an experienced cytologist, and when all relevant clinical data are obtained

Pediatric Solid Pseudopapillary Neoplasm[Spn] of The Pancreas : Case Report and Literature Review

Background: Pediatric pancreatic malignancies are extremely rare with an age population-adjusted incidence of 0.018 cases per 100, 000. In adolescent girls, they usually present as solid pseudopapillary neoplasms[SPNs], low-grade malignant tumors of the exocrine pancreas. Due to their rarity and nonspecific clinical presentation, SPNs are frequently misdiagnosed. The treatment of choice is a complete surgical resection with 5-year survival rate of 97%. Case Report: We report a 17-year-old girl with SPN presenting with jaundice and itchy skin. She was initially misdiagnosed with hepatoblastoma, and treated with neoadjuvant chemotherapy. The girl later underwent surgical excision, and is in complete remission seven years after the diagnosis. Conclusion: Although rare, SPNs need to be included in the differential diagnosis of abdominal masses in children. MRI detected, well-defined, encapsulated, heterogeneous mass, especially in adolescent females, should always be suspicious for SPN. Further prospective multi-institutional studies are required in order to standardize preoperative evaluation and reduce the high misdiagnosis rate

Syringocystadenoma papilliferum: a rare tumor in an unusual location

Objective was to report a very rare case of syringocystadenoma papilliferum, describing its clinical presentation, histopathological appearance and surgical management. An 11-year-old girl presented with a partially lobulated linear plaque on her right lower back present since birth, which was clinically diagnosed at first as linear epidermal nevus. The tumor was surgically removed. The histopathology confirmed the diagnosis of syringocystadenoma papilliferum. Syringocystadenoma papilliferum is a benign adnexal tumor that occurs most often in the head and neck. The tumor has characteristic histological features, and varied and non-distinct clinical findings. Although extremely rare, this lesion should be considered in the differential diagnosis of skin tumors in children

Neonatal Hemophagocytic Lymphohistiocytosis – Case Report

Hemophagocytic lymphohystiocytosis (HLH) represents a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, hepatosplenomegaly, and cytopenias. The most prominent histopathological feature of HLH is an accumulation of activated T lymphocytes and macrophages predominantly in lymphoid tissues. Although it can occur in all age groups, neonatal-onset HLH is very rare. We report on a case of HLH presenting with anemia and respiratory distress at birth. Several weeks prior to diagnosis the symptoms were attributed to a systemic infection. The child developed typical clinical and laboratory findings, and was diagnosed with HLH according to HLH-2004 guidelines. Chemo- -immunotherapy was initiated, but after a temporary control of the disease the patient succumbed to rapidly progressive HLH. Post-mortem, extensive hemophagocytosis was found in multiple organs. No specific genetic defect was identified. HLH is potentially fatal childhood disease. It is important for pediatricians to be able to early identify this disorder and commence the therapy before overwhelming disease activity develops