Ewa Zielińska-Pająk

Coexistence of gastrointestinal stromal tumors with other neoplasms

BackgroundThe purpose of this study was to assess the prevalence of other neoplasms in patients with gastrointestinal stromal tumors (GISTs) and to compare clinical and histopathological data in patients with a GIST and accompanying neoplasms and in patients with GIST only.MethodsThe analysis encompassed 82 patients with a GIST from among 330 300 patients whose surgical specimens, biopsies, and autopsies were evaluated between January 1989 and June 2006. A subgroup of patients with other types of neoplasms was selected.ResultsOther neoplasms in patients with a GIST were diagnosed in 22 of the 82 (26.8%) patients. The most common accompanying neoplasms were colorectal (nine cases) and gastric (four cases) adenocarcinoma, as well as pancreatic adenocarcinoma (three cases). There was a tendency toward more common localization of a GIST in the small intestine in patients with other neoplasms than in patients with a GIST alone (P < 0.09). Tumors with very low risk of aggressive behavior were more frequent in patients with a GIST accompanied by other neoplasms than in the other group (P < 0.05). No phenotypic differences in GIST cells were found between the two groups.ConclusionsIn almost 27% of the study population, GISTs coexisted with other neoplasms. A greater proportion of patients with a GIST localized in the small intestine and/or characterized by a very low risk of aggressive behavior and accompanying other neoplasms, compared with a GIST alone, most likely reflects the fact that in the first group, GISTs tended to be an incidental finding during surgery. The results were affected by patient selection and the type of tissue material available.

Intraductal oncocytic papillary neoplasms of the pancreas and bile ducts: a description of five new cases and review based on a systematic survey of the literature

BackgroundIntraductal oncocytic papillary neoplasms (IOPN) are rare tumors of the pancreatic and biliary ductal system. It is not absolutely clear if the molecular and clinicopathologic characteristics of IOPN differ significantly from other related lesions, namely intraductal papillary mucinous neoplasms (IPMN). Therefore it is not clear if it is reasonable to consider IOPN as a separate diagnostic and clinical entity.MethodsIn order to describe the clinicopathologic characteristics of IOPN and to compare them with the IPMN profile, we performed a systematic review of the literature and additionally studied five previously unreported IOPN cases.ResultsIOPN differ from IPMN by lack of K-ras gene mutations in all studied cases. Several differences in the clinical and biological profile between IOPN and IPMN exist, but they are of quantitative rather than of qualitative nature. Additionally, pancreaticobiliary or gastric-foveolar IPMN components may coexist with IOPN component within a single lesion, which suggests at least a partial relation of the pathogenetic pathways of IPMN and IOPN. Importantly, the pathogenesis of accumulation of mitochondria and oxyphilic appearance of IOPN remains unknown.ConclusionsAt present, there are no reliable criteria other than histopathological picture and K-ras gene status to differentiate IOPN from IPMN. In particular, no clear differences in optimal treatment options and prognosis between these tumors are known. Further studies are needed to clarify the biology of IOPN and to establish their position in clinicopathologic classifications of pancreatic tumors.

Discrepancies between two alternative staging systems (European Neuroendocrine Tumor Society 2006 and American Joint Committee on Cancer/Union for International Cancer Control 2010) of neuroendocrine neoplasms of the pancreas. A study of 50 cases

The aim of our study was to identify and describe potential inconsistencies between two alternative staging systems of pancreatic neuroendocrine neoplasms (pNENs)--the European Neuroendocrine Tumor Society (ENETS) system (2006) and the American Joint Committee on Cancer/Union for International Cancer Control (AJCC-UICC) system (2010). To address this issue, we performed a retrospective clinico-pathological study of 50 cases of pNENs. We found 9 (18%) cases of ENETS/AJCC-UICC discrepancies regarding the primary tumor stage. They included 7 cases of T2/T3 disagreement and 2 cases of T3/T4 disagreement. In addition, we discussed the issue of potential T1/T2 discrepancy (however, we did not observe any such a case). Another inconsistency was related to the application of different stage prognostic groupings between both systems. In conclusion, the discrepancies between ENETS and AJCC-UICC staging systems for pNENs are relatively frequent and heterogeneous. We believe that they should be rigorously recognized. This is necessary for the evaluation of prognostic factors and the effectiveness of therapeutic options used in patients with pNENs.

Different approaches to assessment of lymph nodes and surgical margin status in patients with ductal adenocarcinoma of the pancreas treated with pancreaticoduodenectomy.

Aim: To develop a method of gross examination of pancreaticoduodenectomy specimens with pancreatic ductal adenocarcinoma, allowing adequate assessment of the entire pancreatic surface as a surgical margin, which would not affect the lymph node yield. Methods: We retrospectively compared the R1 rates (i.e., proportions of patients with microscopic residual tumour at surgical margins) and lymph node yield in a series of 67 consecutive cases of pT3 ductal adenocarcinomas diagnosed in pancreaticoduodenectomy specimens during three different periods of time and sampled using three different approaches: (1) period 2006–2007, when the pancreatic surface (except for the transection margin and superior mesenteric artery margin) was not examined; (2) period January–September 2008, when the posterior pancreatic surface (posterior circumferential radial margin) was examined using an improved method based on sampling of 2.0–2.5 mm thick consecutive slices perpendicular to the duodenal axis; and (3) period October 2008 – June 2009, when the whole surface of the pancreatic head was sampled using the approach mentioned above. Results: The R1 rates in three consecutive time periods were 23.5%, 40% and 53.8%, respectively. Median numbers of retrieved peripancreatic lymph nodes were 11.0, 12.0 and 14.0, respectively. Conclusions: The newly proposed approach allowed adequate assessment of the entire pancreatic head surface as a surgical margin and reduced the risk of under‐detection of R1 status. Moreover, this approach did not affect the number of peripancreatic lymph nodes examined.

Mesenchymal hamartoma of the liver imitating gastrointestinal stromal tumor

Mezenchymalne guzy wątroby o utkaniu hamartoma to łagodne guzy o nieznanej etiologii, które najczęściej rozpoznawane są u dzieci do 2. roku życia, natomiast wyjątkowo rzadko u osób dorosłych. Poniżej przedstawiono przypadek 25-letniego chorego z poinfekcyjną marskością wątroby i nadciśnieniem wrotnym, po licznych interwencjach chirurgicznych w obrębie jamy brzusznej, u którego w badaniach obrazowych stwierdzono bogato unaczynioną patologiczną masę w bezpośrednim sąsiedztwie żołądka, budzącą podejrzenie guza stromalnego przewodu pokarmowego. Ze względu na duże ryzyko wystąpienia powikłań w okresie okołooperacyjnym (ryzyko zgonu z powodu marskości wątroby oszacowano na podstawie wskaźnika MELD na 10%) kwalifikację do zabiegu chirurgicznego poprzedziła szczegółowa diagnostyka, która nie potwierdziła choroby rozrostowej. Po wyczerpaniu możliwości diagnostycznych chorego zakwalifikowano do zabiegu chirurgicznego, w czasie którego usunięto z okolicy więzadła wątrobowo-dwunastniczego dobrze ograniczony, spoisty guz o średnicy około 4 cm. W badaniu mikroskopowym materiału pooperacyjnego stwierdzono w guzie obecność mezenchymalnego podścieliska, hepatocytów oraz nabłonka przewodzików żółciowych. Obraz zinterpretowano jako ektopowy mezenchymalny guz wątroby o utkaniu hamartoma. Abstract

Guz Küttnera (chronic sclerosing sialadenitis) – rzadka przyczyna powiększenia ślinianki podżuchwowej ☆

Summary Kuttners tumor is a benign tumour-like lesion of the salivary glands. Predominantly affects the submandibular gland. It is also known as chronic sclerosing sialoadenitis or cirrhosis of submandibular gland. This is an underrecognized entity in the surgical pathology and cytology literature. Most patients experience recurrent pain, discharge and swelling that is often associated with eating, but others only have asymptomatic hard swelling of the submandibular gland. Histologic examination of the excised submandibular glands revealed preserved lobular architecture, thickening of interlobular septa by sclerotic tissue, dense lymphoplasmacytic infiltrate, preservation of ducts with periductal fibrosis, and variable loss of acini. The morphologic appearance, in conjunction with the elevated IgG4 expression, distinguishes chronic sclerosing sialadenitis from other inflammatory diseases of the salivary glands. Chronic sclerosing sialadenitis belongs to the spectrum of IgG4-related diseases. We present a case of Kuttners tumor in a 62-year-old female treated by surgery. Although this disease was first described by Kuttner in 1896, this clinical entity which masquerades as carcinoma is underdiagnosed by many surgeons. There is not enough evidence to support any diagnostic means that could help in the differential diagnosis of this benign condition. Given the high rate of malignancy in firm, painless lesions of the submandibular gland, surgical excision is often advocated and Kuttners tumor is usually diagnosed by the histopathologist.

Grzybica zatok przynosowych – trudności w diagnostyce i leczeniu☆

Summary Introduction The aim of this work was to reveal the problems of diagnostics and treatments of fungal rhinosinisitis. Material and methods Material includes 6 patients (4 women and 2 man, average age 55) treated between years 2005-2010 in the ENT Department of Silesian Medical University in Katowice. All patients were treated by surgery in general anesthesia. All patients had performed CT scan of paranasal sinus before surgical procedures. Removed materials were send to a histological and microbiological examination. Results In the studied material were 5 cases of noninvasive fungal rhinosinusitis (3 case of a fungal rhonosinusitis of the right and 2 of the left maxillary sinus) and 1 case of an invasive fungal rhinosinusitis that coused destroyment of apper and lower wall of the maxillary sinus and penetration to the right orbital. All patients underwent external approach surgery under general anesthesia. All of examinations the histological and microbiological confirmed fungal etiology (Aspergillus). A possible factor in the development of fungal sinusitis in one patient was previously treated pulmonary aspergillosis, but in a patient with invasive fungal sinusitis probably played a role in type 2 diabetes and old age. Pharmacotherapy included in a patient with invasive fungal sinusitis (Amphotericin B ® ) and in a patient with a history of lung Aspergillozie (Orungal ® ). The results of the control CT scan obtained full recovery. Conclusion Fungal infection of paranasal sinuses is a problem located at the intersection of many medical specialties. Force look at the patient in a multidisciplinary. Do not forget that it may be the final diagnosis as well as a symptom of immune deficiency, or metabolic disorders.