Eosinophilic Gastroenteritis: An Underdiagnosed Condition.

BACKGROUND: Eosinophilic gastroenteritis (EOGE) is a rare idiopathic disease characterized by eosinophil-predominant inflammation of the stomach and/or intestines. Our aims are to determine the epidemiology, clinical features and outcomes of EOGE cases in a tertiary-care hospital. METHODS: Retrospective cohort study of patients with gastrointestinal eosinophilic infiltration from 2004 through 2014. All relevant specimens were reviewed by an expert pathologist. Significant eosinophilic infiltrate was defined as >25 eosinophils/HPF in the stomach or small intestine and >50 eosinophils/HPF in the colon. RESULTS: Three hundred and sixty-one charts were reviewed and 13 EOGE cases were identified, including nine adults and four pediatric cases. The majority (78 %) of adult cases were females. Clinical presentation was variable; most patients (62 %) had abdominal pain, followed by diarrhea (31 %) and nausea/vomiting (31 %). Atopy and food allergies were present in 54 and 38 % of patients, respectively. Weight loss and failure to thrive were present only in pediatric cases (50 vs 0 %; P = .01). Most EOGE cases (69 %) had peripheral eosinophilia, which was more prominent in patients with ascites compared to patients without ascites (37.3 ± 25.4 vs 9.3 ± 5.4 %; P = .01). Among patients who had long-term follow-up; 30 % had spontaneous remission, 60 % responded to steroids and/or restriction diet, and 10 % had refractory disease. CONCLUSION: EOGE is an underdiagnosed condition. In contrast to eosinophilic esophagitis; the disease might be female-predominant in adults. High index of clinical suspicion is required for diagnosis. Further studies about the long-term outcomes and the efficacy of restriction diet in adult patients are required.

Selective staining of gastric biopsies for H. pylori does not affect detection rates or turnaround time and improves cost compared to reflexive staining.

AIMS: To evaluate how reflexive versus selective H. pylori stains affect detection rates, turnaround time (TAT), and cost savings in a real life practice environment following an institutional policy change. METHODS: The aforementioned parameters were evaluated in all cases in the year preceding and the year following an institutional policy change from reflexive to selective staining. RESULTS: 1497 patients comprised the reflexive stain (RS) group of which 228 (15.2%) were H. pylori positive. 1629 patients comprised the selective stain (SS) group of which 237 (14.5%) were H. pylori positive. There was no significant difference in H. pylori detection rates between the RS and SS groups (OR=0.95, 95% CI=0.78-1.15, p=0.59). TATs were similarly equivalent with a mean of 52.4h for the RS cohort and 53.7h for the SS cohort (p=0.344), both of which included a resident preview day. We calculated an average laboratory cost savings of $11.68 per case, which saved our department over $15,000 (37%) in the year following the policy change. CONCLUSIONS: Our results support a policy of selective staining for H. pylori as opposed to reflexive staining and go on to show that laboratories that change their policy can expect to generate cost savings without compromising detection rates or TAT.

A morphologic reappraisal of endoscopically but not histologically apparent polyps and the emergence of the overlooked goblet cell--rich hyperplastic polyp.

Goblet cell--rich hyperplastic polyps (GCRHP) are morphologically subtle compared to microvesicular hyperplastic polyps (MVHP) and are believed to be the most commonly unrecognized serrated polyp, though this has not been systematically studied. We hypothesize that a gastrointestinal pathologist's review of endoscopically but not histologically apparent polyps will identify previously missed GCRHPs, a finding that may be clinically significant if the addition of this subtype of serrated polyp contributes to sufficient numeric criteria for a clinical diagnosis of serrated polyposis syndrome (SPS). Two blinded reviews were performed on 160 endoscopically but not histologically apparent polyps by a gastrointestinal pathologist, separated by a 6 month "washout period." A final review diagnosis of GCRHP was applied to all polyps with complete agreement on both reviews. Patient records were then searched to determine if the addition of a GCRHP resulted in sufficient numeric criteria for a clinical diagnosis of SPS. Fourteen (9%) polyps were reclassified as GCRHPs. The majority (n = 12, 86%) were originally called "colonic mucosa with surface hyperplastic change (CMWSHC)." Two polyps (1%) were re-classified as MVHPs. No other serrated or adenomatous polyps were identified. For each patient, the addition of a hyperplastic polyp did not result in a clinical diagnosis of SPS, though one patient fell short of this diagnosis by only one polyp. GCRHPs are the most commonly underdiagnosed serrated polyp and are often called CMWSHC. The addition of previously missed GCRHPs is unlikely to contribute to a diagnosis of SPS in an individual patient.

Performing colonic mast cell counts in patients with chronic diarrhea of unknown etiology has limited diagnostic use.

CONTEXT: Mastocytic enterocolitis is a recently described entity defined by chronic diarrhea of unknown etiology and normal colon biopsy results with increased mast cells (MCs) seen on special stains. These patients may benefit from mast cell stabilizers; however, the clinical utility of MC counts remains unknown. OBJECTIVE: To determine the clinical utility of colonic MC counts on normal biopsies in patients with chronic diarrhea of unknown etiology. DESIGN: Blinded MC counts using a c-Kit stain were performed in 76 consecutive patients with chronic diarrhea of unknown etiology who had normal colon biopsy results and in 89 consecutive control patients presenting for screening colonoscopy. Mast cells were counted per single high-power field in the highest-density area. A t test was used to compare the counts, and receiver operating characteristic curves were generated to examine sensitive and specific cutoff values. RESULTS: Overall, MC counts averaged 31 MCs per high-power field in the study group versus 24 MCs per high-power field in the control group (P < .001). When stratified by location, a significant increase was seen in biopsies from the left colon only. Receiver operating characteristic analysis revealed that overall MC counts, left-sided MC counts, and the difference between right- and left-sided MC counts did not yield discriminatory cutoff values (area under the curve, 0.68, 0.74, and 0.81, respectively). CONCLUSIONS: Mast cell counts were increased in patients with chronic diarrhea of unknown etiology, primarily in the left colon. However, receiver operating characteristic analysis demonstrates no discriminatory cutoff values. Quantitative MC stains yield little useful diagnostic information, and further studies are necessary to determine whether mastocytic enterocolitis truly represents a distinct entity.

Synchronous adrenocortical neoplasms, paragangliomas, and pheochromocytomas: syndromic considerations regarding an unusual constellation of endocrine tumors.

The most common clinical syndromes presenting with paragangliomas and/or pheochromocytomas as their endocrine components are multiple endocrine neoplasia type 2, neurofibromatosis, Von Hippel-Lindau syndrome, Carney-Stratakis syndrome, Carney triad, and the recently described hereditary paraganglioma syndrome. Only Carney triad is known to also present with adrenocortical adenomas, currently representing the only described syndrome in which all 3 of the aforementioned tumors are found together. In most cases, prototypical lesions of the triad such as gastrointestinal stromal tumor and pulmonary chondromas are also seen. We present a case of a young woman with synchronous paragangliomas, adrenal/extra-adrenal cortical neoplasms, and pheochromocytoma without genetic mutations for multiple endocrine neoplasia 2, Von Hippel-Lindau syndrome, neurofibromatosis, and succinate dehydrogenase. We speculate that this represents a previously undescribed presentation of Carney triad and, at the very least, indicates the need for monitoring for the development of other tumors of the triad.

Squamoid cyst of pancreatic ducts: a case series describing novel immunohistochemistry, cytology, and quantitative cyst fluid chemistry.

Squamoid cyst of pancreatic ducts (SCPD) is a benign pancreatic cyst often misdiagnosed preoperatively as a mucinous cyst. The histopathologic features are well described but the cytology and quantitative fluid chemistry profiles from fine-needle aspiration have not been reported. This case series discusses the cytology and cyst fluid chemistry profiles in 2 SCPDs and describes morphologic and immunohistochemical features that have not been previously reported. Fine-needle aspiration of 2 SCPDs yielded acellular debris lacking mucin or exfoliated squamous cells. Two cysts had elevated fluid carcinoembryonic antigen (CEA) and amylase levels. Positive immunohistochemical staining included cytokeratin 5/6, pCEA, synaptophysin, and chromogranin (both focal). MUC2 and MUC5AC showed negativity in all cases, while PAX8 showed negative nuclear staining. An accurate preoperative diagnosis of SCPD is potentially difficult in the setting of elevated fluid CEA levels, and acellular cytology as a mucinous cyst cannot be confidently excluded.

Radiology estimates of viable tumor percentage in hepatocellular carcinoma ablation cavities correlate poorly with pathology assessment.

CONTEXT: No study has evaluated radiology/pathology correlation of percentage viable tumor (PVT) estimates in ablated hepatocellular carcinoma (HCC) to examine the reliability of radiologic estimates. OBJECTIVE: To determine how well interdisciplinary PVT estimates correlate and identify pathologic factors that influence this correlation. DESIGN: Pathologists and radiologists established blinded PVT estimates in 22 HCC ablation cavities. Paired sample t tests examined the differences between the interdisciplinary estimates. RESULTS: Fifteen cavities had pathologic viable tumor (VT) (68%) and 6 had radiographic VT (22%). Radiology's sensitivity for detecting VT was 40% and the specificity was 100%. Pathology detected significantly more VT than radiology (pathology mean = 22.3% versus radiology mean = 2.6%; P = .005). Five cavities had tumor growth in a discontinuous rim pattern, 7 in a nodular pattern, and 3 in a solid pattern. Radiology did not detect VT in cavities with a discontinuous rim pattern (sensitivity = 0%) but did detect VT in 3 cavities with a nodular pattern (sensitivity = 43%), and in all cavities with a solid pattern (sensitivity = 100%). There was no significant difference in PVT estimates in cavities 3.5 cm or larger (P = .07), but there was a significant difference in cavities smaller than 3.5 cm (P = .01). CONCLUSION: This study clarifies that the risk of underestimation by imaging is greatest in small lesions (<3.5 cm), though the sensitivity of detection depends primarily on the tumor growth pattern within the cavity. This underestimation raises the question of whether basing treatment decisions on a radiologic impression of complete ablation is valid.

Adult testicular granulosa cell tumor: a review of the literature for clinicopathologic predictors of malignancy.

Adult testicular granulosa cell tumors are rare sex cord-stromal tumors of which only 28 have been previously reported. As compared with their ovarian counterparts, these tumors may follow a more aggressive course because the proportion of malignant cases is higher. To date, there are no clinical or pathologic features that definitively predict malignancy. We reviewed all prior case reports for features that may predict their malignant potential. Tumor size greater than 5.0 cm is the only feature statistically associated with malignancy. Mitotic count, tumor necrosis, patient age, and the presence of gynecomastia do not, at present, predict benign versus malignant behavior.

Small-intestinal rhabdoid gastrointestinal stromal tumor (GIST): mutation analysis and clinical implications of a rare morphological variant.

Rhabdoid features in gastrointestinal stromal tumors (GISTs) are rare. To the authors' knowledge, only 51 cases have been reported. Most of these reports consist of case series in which the rhabdoid GISTs comprise a small proportion of the tumors studied. Information regarding site of origin and clinical behavior is sparse. Although the stomach is the only site of origin documented, most reports do not include this data. Malignancy has not been reported, though follow-up is inadequate in most cases to comment on tumor behavior. Exon 11 mutations comprise all previously described KIT mutations, the majority of which are deletions. The authors present the case of a malignant small-intestinal rhabdoid GIST that recurred twice following resection and treatment with tyrosine kinase inhibitors. The tumor harbored a KIT exon 11, 579-580 LY insertion that, to the authors' knowledge, has not been previously reported. This case is the first rhabdoid GIST described in the small intestine and is the first to show documented evidence of malignancy.