Gastric Perineurioma: A Case Report and Review of the Literature.

Perineuriomas of the gastrointestinal tract, formerly known as benign fibroblastic polyps, most commonly occur as polyps on screening colonoscopy, particularly in the distal colon. Gastric examples are exceedingly rare. We report the sixth patient with a gastric perineurioma in a 57-year-old female. Histologically, the lesion was composed of bland spindle cells without cytologic atypia or mitotic activity located in the gastric lamina propria. The spindled cells were strongly positive for GLUT1 and focally reactive for epithelial membrane antigen (EMA). The morphologic and immunophenotypic findings were those of gastric perineurioma.

Along for the Ride: Intrahepatic Cholangiocarcinoma with Concomitant LECT2 Amyloidosis.

We present a case of a 69-year-old Hispanic male with a past medical history of type II diabetes mellitus who presented with a two-month history of abdominal pain. A CT scan was performed which identified a liver mass. Biopsy of the liver mass revealed infiltration of normal liver parenchyma by atypical glands surrounded by pale eosinophilic material. The atypical glands were positive for CK7, while negative for CK20, CDX-2, and TTF-1, consistent with intrahepatic cholangiocarcinoma. A Congo red stain was performed, which highlighted salmon-orange areas, some with a globular appearance, around the glands and within the sinusoids and vasculature. Under polarized light, these areas displayed apple-green birefringence. These findings were consistent with amyloidosis, which was further supported by identification of ALECT2- (leukocyte chemotactic factor-2-) type amyloid on mass spectrometry. To our knowledge, this is the first documented case of intrahepatic cholangiocarcinoma arising in association with LECT2 amyloidosis.

Mixed Acinar-Endocrine Carcinoma (MAEC) Arising in Duodenal Pancreatic Heterotopia.

Mixed acinar-endocrine carcinoma (MAEC) of the pancreas is a rare neoplasm, consisting of at least 25%-30% of acinar and neuroendocrine populations. Patients are often middle-aged and present with nonspecific symptoms. Imaging typically reveals a solid lesion in the pancreatic head. Management involves surgical resection and the overall prognosis is variable. Here, we present a case of a 48-year-old male who presented with a MAEC arising from duodenal pancreatic heterotopia. This is the one of the first cases, with histologic evidence, of MAEC arising from pancreatic heterotopia.

Routine Hematoxylin and Eosin Stain Is Specific for the Diagnosis of Cytomegalovirus Infection in Gastrointestinal Biopsy Specimens.

BACKGROUND: Gastrointestinal cytomegalovirus (CMV) infection is a serious complication in immunocompromised patients; clinicians often expect expedited results for biopsy specimens. Our goal is to determine the accuracy of identification of CMV on hematoxylin and eosin (H&E) stain. METHODS AND RESULTS: A total of 361 biopsy specimens from 273 patients with suspicion for CMV infection were retrieved. CMV was detected by immunohistochemistry (IHC) in 37 specimens acquired from 33 individual patients (average age = 54 years). Among the CMV-positive patients, 29 (88%) were reported to be immunosuppressed. Colon was the most common affected location. Of 37 CMV-positive specimens by IHC, 28 were positive by H&E (76%), 6 were negative (16%), and 3 were suspicious (8%). Of the 29 positive specimens on H&E, 28 were confirmed by IHC (97%) and 1 was indeterminate (3%). The sensitivity and specificity of H&E were 84% and 94%, respectively; the positive predictive value was 97%, and the negative predictive value was 93% ( P < .00001). CONCLUSION: Our results show that a preliminary diagnosis of CMV infection, based on H&E stains, can be reported with high specificity and low risk for false-positive results. Suspicious cases should be deferred pending the result of IHC stains.

Pulse Granulomas of the Gastrointestinal Tract and Gallbladder: Report of Five Cases.

Hyaline rings with admixed multinucleated giant cells characterize pulse granulomas; the term pulse refers to edible seeds of legume vegetables. The etiology has been controversial, with theories including vascular degenerative changes or a reaction to vegetable material; ultrastructural studies and experimentally induced lesions in animals favor the latter. This lesion is typically seen in the oral cavity, with only rare reports in the gastrointestinal tract and gallbladder. We herein describe five cases of pulse granulomas identified in these sites. All cases contained foreign-body giant cells and vegetable debris within or near hyaline rings. Pulse granulomas may form mass lesions but are usually an incidental finding on microscopic examination. In incidentally detected cases, recognition of pulse granulomas can suggest a mural abscess, fistula, or perforation of the gut, findings which may not be grossly apparent. The presence of vegetable material in all five cases further supports an exogenous pathogenesis.

Histological evaluation in ulcerative colitis.

This review summarizes diagnostic problems, challenges and advances in ulcerative colitis (UC). It emphasizes that, although histopathological examination plays a major role in the diagnosis and management of UC, it should always be interpreted in the context of clinical, endoscopic, and radiological findings. Accurate diagnosis requires knowledge of the classic morphological features of UC, as well as a number of atypical pathological presentations that may cause mis-classification of the disease process, either in resection or biopsy specimens. These atypical pathological presentations include rectal sparing and patchiness of disease at initial presentation of UC in pediatric patients or in the setting of medically treated UC, cecal or ascending colon inflammation in left-sided UC, and backwash ileitis in patients with severe ulcerative pancolitis. Loosely formed microgranulomas, with pale foamy histiocytes adjacent to a damaged crypt or eroded surface, should not be interpreted as evidence of Crohn's disease. Indeterminate colitis should only be used in colectomy specimens as a provisional pathological diagnosis. Patients with UC are at risk for the development of dysplasia and carcinoma; optimal outcomes in UC surveillance programs require familiarity with the diagnostic criteria and challenges relating to UC-associated dysplasia and malignancy. Colon biopsy from UC patients should always be evaluated for dysplasia based on cytological and architectural abnormalities. Accurate interpretation and classification of dysplasia in colon biopsy from UC patients as sporadic adenoma or UC-related dysplasia [flat, adenoma-like, or dysplasia-associated lesion or mass (DALM)] requires clinical and endoscopic correlation. Isolated polypoid dysplastic lesions are considered to be sporadic adenoma if occurring outside areas of histologically proven colitis, or adenoma-like dysplasia if occurring in the diseased segment. Recent data suggest that such lesions may be treated adequately by polypectomy in the absence of flat dysplasia in the patient. UC patients with DALM or flat high-grade dysplasia should be treated by colectomy because of the high probability of adenocarcinoma. The natural history of low-grade dysplasia (LGD) is more controversial: while multifocal LGD, particularly if detected at the time of initial endoscopic examination, is treated with colectomy, unifocal flat LGD detected during surveillance may be managed by close follow-up with increased surveillance. The surveillance interval and treatment options for UC patients with dysplasia are reviewed in detail.

Cholestatic liver injury associated with dietary supplements: a report of five cases in active duty service members.

The use of dietary supplements (DS) is common in the active duty population, often without physician knowledge or approval. DS have been associated with drug-induced liver injury, with rare cases resulting in liver failure or death. We report five cases of transient drug-induced liver injury temporally associated with the use of a total of six DS in active duty service members. All patients presented with elevated serum bilirubin and liver-associated enzymes: three patients had a cholestatic liver enzyme pattern, one had a hepatocellular pattern, and one had a mixed pattern. In all cases, percutaneous needle core biopsies of the liver were obtained and demonstrated a cholestatic pattern of injury with variable periportal fibrosis. Causality was considered highly probable for three cases, probable for one case, and possible for one case. Hepatotoxicity has been previously associated with four of the supplements in our cases. For the two remaining supplements, C4 Extreme and Animal Stak, we are unaware of any previous reports of hepatotoxicity. Health care professionals, in particular military physicians, should be aware of the potential risk of these supplements and be prepared to discuss these risks with their patients.

Esophageal carcinoma cuniculatum: report of 9 cases.

Carcinoma cuniculatum, a unique variant of well-differentiated squamous cell carcinoma, has been only rarely reported in the esophagus. We report 9 cases of esophageal carcinoma cuniculatum diagnosed on esophagectomy specimens in 7 men and 2 women during a 20-year period. All but 1 of the patients presented with persistent or progressive dysphagia. All patients had an esophageal mass or lesion on endoscopic examination. In 8 cases (88.8%), the tumor was located in the distal esophagus. Burrowing was noted on the tumor surface in 2 recent cases on macroscopic examination. All carcinomas were invasive either at the mucosa (n=2), submucosa (n=1), muscularis propria (n=4), adventitia (n=3), or adventitia and lung (n=1). All carcinomas demonstrated a common histologic pattern characterized by hyperkeratosis, acanthosis, dyskeratosis, abnormal keratinization, keratin-filled cyst/furrows, koilocyte-like cells, intraepithelial neutrophils, and focal cytologic atypia. In situ hybridization for human papillomavirus subtypes was negative in all 10 tumors tested. None of the cases showed lymph node metastasis. Two patients died postoperatively due to complications. The remaining patients were followed up for a median duration of 84 months (48 to 214 mo). During the follow-up period, 3 patients died 49, 66, and 214 months after esophagectomy at the ages of 66, 68, and 91 years, respectively; death in these 3 cases was not related to recurrence/metastases of esophageal cancer. Four patients were alive without disease at 48, 49, 84, and 87 months after curative resection. Our report identifies a common histomorphologic pattern of esophageal carcinoma cuniculatum and supports the fact that surgical resection of the tumor by esophagectomy provides long-term survival even in patients with T3 tumor.

Transmural inflammation is not pathognomonic for Crohn's disease of the pouch.

BACKGROUND: Transmural inflammation shown by imaging and histology has been considered a hallmark of Crohn's disease (CD). However, the diagnostic and prognostic value of this feature in CD of the pouch has not been evaluated. This study aimed to evaluate the clinical utility of transmural inflammation in patients with ileal pouch-anal anastomosis (IPAA) using in vivo optical coherence tomography (OCT) and histopathology. METHODS: All the patients were recruited from the subspecialty Pouchitis Clinic. The study consisted of two parts: (1) a prospective study with in vivo through-the-scope OCT for the evaluation of transmural disease in patients with normal or diseased pouches and (2) a retrospective pathology re-review for transmural inflammation in excised pouch specimens of CD and chronic pouchitis. RESULTS: This prospective OCT study enrolled 53 patients: 11 (20.8%) with normal pouches or irritable pouch syndrome, 10 (18.9%) with acute pouchitis, 11 (20.8%) with chronic antibiotic-refractory pouchitis (CARP), and 21 (39.6%) with CD of the pouch. Transmural inflammation, characterized by the loss of layered structure on OCT, was detected in 16 patients (30.2%): 4 with chronic pouchitis and 12 with CD of the pouch. None of the patients with normal pouches, irritable pouch syndrome, or acute pouchitis had transmural disease shown on OCT. Of the 26 patients with pouch failure who had pouch excision, the surgical specimens showed transmural disease in 30% of the CARP patients (3/10) and 12.5% (2/16) of those with CD of the pouch. CONCLUSIONS: Transmural disease in the setting of IPAA is not pathognomonic of CD. Transmural inflammation shown by imaging or histopathology was seen in both CD and CARP. Transmural inflammation of the pouch appeared to be associated with poor pouch outcome.

Immunohistochemical evaluation of androgen receptor, HER-2/neu, and p53 in benign pleomorphic adenomas.

CONTEXT: Immunohistochemical stains for androgen receptor (AR), HER-2/neu, and p53 are used as diagnostic markers associated with malignancy in several histologic types of salivary gland tumors. These markers may be useful in differentiating pleomorphic adenoma with cytologic atypia from intracapsular carcinoma ex pleomorphic adenoma (CXPA), as these tumors are often difficult to distinguish on the basis of morphology alone. OBJECTIVE: To determine whether AR, HER-2/neu, and p53 expression can be seen in entirely benign pleomorphic adenomas. DESIGN: Androgen receptor, HER-2/neu, and p53 immunoreactivity was assessed in 41 histologically and clinically benign pleomorphic adenomas. RESULTS: A total of 3 of 41 pleomorphic adenomas exhibited multifocal areas with moderate staining for HER-2/ neu and AR. The positive staining was mainly confined to the epithelial component, where the ductal epithelium showed no cytologic atypia. Immunoreactivity for p53 was observed in the epithelial component of 5 of 41 cases, none of which stained for HER-2/neu and AR. Mean mitotic rate and Ki-67 index were 1 per 10 high-powered fields and 2.7% in HER-2/neu- and AR-positive cases and 1 per 10 high-powered fields and 2.2% in p53-positive cases. CONCLUSIONS: HER-2/neu, AR, and p53 are expressed in a subset of histologically and clinically benign pleomorphic adenomas. These markers cannot be used to reliably predict early carcinomatous transformation in pleomorphic adenoma.

Pathologic characteristics of solitary small renal masses: can they be predicted by preoperative clinical parameters?

We studied the pathologic features and whether clinical features could predict pathologic outcomes in small renal masses. The study included all adult patients with solitary, nonmetastatic renal masses 4 cm or smaller confirmed by nephrectomy or needle biopsy between 2004 and 2006. Tumor size, histologic type, Fuhrman nuclear grade, and stage were obtained from surgical pathology reports. Clinical variables included age, sex, tumor size, and symptomatology. The study included 290 men and 198 women (mean age, 59.3 years). Median tumor size was 2.6 cm (range, 0.5-4.0 cm). Approximately 84% of masses were incidentally detected. Nonneoplastic entities, benign neoplasms, and low- and high-grade carcinoma accounted for 1.6%, 18.0%, 49.0%, and 31.4% of masses, respectively. Women were more likely to have a benign mass (27.3% vs 14.5% of men, P < .001). Age (P = .56), tumor size (mean, 2.63 vs 2.46 cm for benign; P = .08), and symptomatology (P = .46) were not associated with malignancy. Multivariate analyses using sex, age, tumor size, and symptomatology failed to produce a model useful to predict the pathology of individual tumors. This inability may argue for an increased role for needle biopsy in their evaluation.