Dariusz Gołka

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.

Colloid carcinoma of the pancreas: review of selected pathological and clinical aspects

&NA; Colloid carcinoma (CC) of the pancreas is a histopathological variant of ductal adenocarcinoma, which is characterised by the presence of large pools of extracellular mucin, containing neoplastic cells. The mucin component comprises at least 50% of CC (according to the definition by the World Health Organization) or at least 80% of the tumour (according to the US Armed Forces Institute of Pathology). In the vast majority of cases, CC develop from pre‐existing intraductal papillary mucinous neoplasms, especially those forming intestinal‐type papillae and characterised by MUC2 expression. Data concerning the long‐term prognosis in patients with CC are discrepant. In this review, the authors present contemporary definitions of CC, issues of its epidemiology, symptomatology, pre‐operative diagnostics, histopathology, treatment and prognosis. Special attention has been paid to pathogenesis of CC.

Primary Malignant Fibrous Histiocytoma of the Heart

A 72-year-old male diagnosed with heart failure and dyspnea was found to have a lesion in his right atrium. Histological examination of the tumor revealed it to be a malignant fibrous histiocytoma. The patients management and subsequent follow-up are presented.

Serous neoplasms of the pancreas share many, but not all aspects of their microvascular and angiogenic profile with low-grade clear cell renal cell carcinomas

Similarly to clear cell renal cell carcinomas (CCRCC), serous neoplasms (SN) of the pancreas frequently show inactivation of VHL gene, clear cell histology and abundant microvasculature. Data on the microvascular and angiogenic profile of SN are scarce. Aiming to examine further the striking resemblance of clear cell epithelial neoplasia in pancreas and kidney, we compared the microvascular profile and expression of pro-angiogenic factors in SN and in CCRCC using immunohistochemical stains. SN and CCRCC shared a predominance of differentiated blood vessels, scarcity of lymphatic vessels, presence of CD105 and claudin-5 in tumoral vessels, expression of vascular endothelial growth factor (VEGF)-A, cyclooxygenase-2 (COX-2), carbonic anhydrase IX in tumoral cells, and lack of VEGF-C in tumoral cells. In contrast to CCRCC, SN showed lower pericyte coverage of vessels, lower blood vessel endothelial cell proliferaction fraction, more pronounced VEGF receptor (VEGFR)-2 and glucose transporter-1 expression, higher inducible (iNOS) but lower endothelial nitric oxide synthase (eNOS) expression, as well as presence of VEGFR-3 and D2-40 expression in epithelial cells. In conclusion, we found a significant similarity but not equality of microvascular biology of SN and CCRCC. We recognized VEGFR-2, VEGFR-3, COX-2, iNOS, eNOS and D2-40 as new markers of epithelial cells of SN of the pancreas.

MUC1 immunoexpression is a virtually constant feature of clear cell renal cell carcinoma metastatic to the pancreas.

To the Editor: We read with great interest a review paper of Dr Sangoi and coauthors on the usefulness of immunohistochemical stains in the diagnosis of metastatic clear cell renal cell carcinoma (CC-RCC) published in one of the recent issues of Advances in Anatomic Pathology.1 We really appreciate this paper and previous works of the authors2–4 regarding the issue of CCRCC. We find these contributions very useful in our diagnostic practice as they contain well-balanced and evidencebased practical recommendations. However, we would like to draw attention of the authors and the readers to 1 selected aspect of diagnosis of metastatic CC-RCC described in the aforementioned review, namely the relevance of application of MUC1 immunostain in differential diagnostics of neoplasms of the pancreas composed of clear cells. Authors of the review stated1 that MUC1 [along with carcinoembryonic antigen (CEA) and cytokeratin 7 (CK7)] may be used to distinguish between metastatic CC-RCC of the pancreas and primary pancreatic ductal adenocarcinoma (PDAC), as MUC1 is “not typically immunoreactive in CCRCC,” in contrast to PDAC. We have found a similar statement on CEA and MUC1 (but not CK7) in CC-RCC in the AFIP reference book on pancreatic neoplasms (p. 328).5 Primary publications, which Dr Sangoi and coauthors and the authors of the AFIP book refer to (Adsay et al,6 Luttges et al,7 and Adsay et al,6 respectively), are devoted to PDAC with clear cells but not to CC-RCC metastatic to the pancreas itself. As in our anecdotal experience metastatic CC-RCC of the pancreas may in fact be MUC1-positive, we reviewed the literature on that issue and performed an immunohistochemical study on a series of CC-RCC metastatic to the pancreas using 2 commercially available MUC1 antibodies. Metastatic CC-RCC represents a complicated clinical issue with many questions yet unanswered.8,9 The diagnosis of CC-RCC metastatic to the pancreas versus PDAC in a biopsy or TABLE 1. The Clinicopathologic Characteristics of Cases of Clear Cell Renal Cell Carcinomas Metastatic to the Pancreas Included in This Study