Intramural Nodules Research Articles

Xanthogranulomatous cholecystitis: the use of preoperative CT findings to differentiate it from gallbladder carcinoma

A retrospective analysis was performed on 32 patients with histologically confirmed xanthogranulomatous cholecystitis (XGC) and 21 patients with gallbladder carcinoma who underwent surgical treatment between 1998 and 2007. All patients underwent preoperative CT scanning. The CT features analyzed were: the presence of intramural hypoattenuated nodules or bands, mucosal line, the patterns of wall thickening and enhancement, and the presence of stones in the gallbladder. The variables of the CT findings with XGC were analyzed using multivariate logistic regression analysis. Intramural hypoattenuated nodules were observed in 21 patients (65%) with XGC, but in only six patients (29%) with gallbladder carcinoma (P < 0.01). The mucosal line was observed in 27 patients (84%) with XGC and in only four patients (19%) with gallbladder carcinoma (P < 0.0001). Gallstones were noted in 24 patients (75%) with XGC and five patients (24%) with gallbladder carcinoma (P < 0.001). There was no significant difference in the pattern of gallbladder wall thickening (diffuse or focal) and the presence of changes outside the gallbladder. Multivariate logistic regression analysis revealed from the CT features that the enhanced continuous mucosal line (P = 0.0013) and the presence of gallstones (P = 0.0072) were independently correlated with XGC. CT features of the enhanced continuous mucosal line in a thickened gallbladder wall, together with gallstones in a patient with chronic gallbladder disease, are highly suggestive of XGC. Accurate diagnosis of XGC may therefore indicate the need to select a less aggressive surgical approach.

Ultrasonographic findings and natural history of intraductal papillary-mucinous neoplasms of the pancreas.

It is clear that the prevalence of malignancy is high in the main-duct type of intraductal papillary-mucinous neoplasm (IPMN). Branch-duct IPMNs include several histologic conditions such as carcinoma, adenoma, and hyperplasia. Intraductal papillary adenocarcinoma and papillary adenoma are characterized by papillary protrusions and thick septum-like structures in dilated ducts as delineated by ultrasonography. A solid mass showing a mixedecho pattern in the pancreatic parenchyma is a characteristic finding of invasive types of IPMN. The international guidelines for the management of branch-duct IPMNs suggest that the appearance of symptoms attributable to the cyst, the presence of intramural nodules, a cyst size greater than 30 mm, and dilation of the main pancreatic duct (>6 mm) are indications for resection. Based on the relationship between the height of a papillary protrusion and the diameter of a cystic dilated branch as well as on histological findings, branch-duct IPMNs with papillary protrusions more than 10 mm in height as shown by imaging should be resected, and it is not adequate to differentiate carcinoma from other lesions based on the diameter of cystic branches alone. A follow-up study on branch-duct IPMNs revealed that most papillary protrusions showed a slow increase in size or development of lateral spread, and that there was no development of cancer with stromal invasion during an average follow-up of 46 months. Therefore, the presence of intramural nodules alone should not be an indication for surgery. Also, patients without papillary protrusions or thick septum-like structures are not immediate candidates for surgery. Invasive adenocarcinoma can develop at a pancreatic site different from the area of interest showing cystic changes, with such invasion possibly being multicentric. Therefore, in patients with branch-duct IPMNs, attention should be paid to the entire pancreas when performing follow-up examinations.

Pancreatic-duct–lavage cytology in candidates for surgical resection of branch-duct intraductal papillary mucinous neoplasm of the pancreas: should the International Consensus Guidelines be revised?

The International Consensus Guidelines are helpful for the management of branch-duct intraductal papillary mucinous neoplasms (IPMNs), because they allow us to exclude malignancy. However, it is not possible to predict malignancy with certainty, and further preoperative differentiation between benign and malignant IPMNs is required to avoid the false-positive results. To examine the usefulness of pancreatic-duct-lavage cytology by using an originally designed double-lumen catheter for discriminating benign and malignant IPMNs of the branch-duct type in candidates for surgical resection based on the International Consensus Guidelines. Pancreatic-duct-lavage cytology was investigated in 24 patients with branch-duct IPMNs who underwent surgical resection based on the International Consensus Guidelines, namely, they either had intramural nodules or the ectatic branch duct was >30 mm in diameter. Single-center retrospective study. Academic medical center. The sensitivity and specificity of pancreatic-duct-lavage cytology for discriminating benign from malignant IPMNs. More than 30 mL of pancreatic-duct-lavage fluid was obtained from each patient, and there were no patients with noninformative results. The sensitivity, specificity, positive predictive value, and negative predictive value of the cytologic diagnosis were 78%, 93%, 88%, and 88%, respectively. Single-center and small number of patients. Pancreatic-duct-lavage cytology can improve differentiation between benign and malignant IPMNs of the branch-duct type in candidates for surgical resection based on the International Consensus Guidelines.

Do Consensus Indications for Resection in Branch Duct Intraductal Papillary Mucinous Neoplasm Predict Malignancy? A Study of 147 Patients

Recent consensus guidelines suggest that presence of > or =1 of the following is an indication for resection (IR) of branch duct intraductal papillary mucinous neoplasm (IPMN-Br): cyst-related symptoms, main pancreatic duct diameter > or =10 mm, cyst size > or =30 mm, intramural nodules, or cyst fluid cytology suspicious/positive for malignancy. Among a cohort of patients with IPMN-Br we determined if the consensus IR (CIR), presence of multifocal IPMN-Br, or growth of cyst size on follow-up predict malignancy. We identified 147 patients with IPMN-Br of whom 66 underwent surgical resection at diagnosis and 81 were followed conservatively, of whom 11 were resected during follow-up. Clinical, imaging, histological, and cyst fluid characteristics from all 147 patients with IPMN-Br were obtained from clinical records and/or by contacting the patients. In all cases, presence of CIR at baseline and during follow-up (N = 66), presence of multifocal cysts (N = 57), and increase in cyst size (N = 38) were noted. Among the 77 resected IPMN-Brs, at initial evaluation 61 had at least one CIR and 16 had none. Malignancy was present in 9/61 (15%) with CIR and 0/16 without IR (P= 0.1). When presence of any one of the CIR was taken as an indicator of malignancy, the CIR had a sensitivity, specificity, positive predictive value, and negative predictive value of 100%, 23%, 14%, and 100%, respectively. Prevalence of malignancy in those with single versus multifocal IPMN-Br was similar (13%vs 11%). No patient has developed malignancy after a median follow-up of 15 months. So far, none of the 38 patients with increase in cyst size on follow-up has developed malignancy related symptoms. Suggested consensus indications for resection identify all patients with malignancy; however, their specificity is low. In the short term it would be safe to follow patients without these features.

An unusual cause of cholecystitis: Heterotopic pancreatic tissue in the gallbladder

Gallbladder localization of heterotopic pancreas (HP) is uncommon and very rarely gives rise to symptoms. Herein we report a case of HP found in the gallbladder neck presented with signs and symptoms of cholecystitis. The patient was a 40-year old male, suffering from epigastric pain, abdominal fullness and fever. On physical examination, the right upper abdomen was tender with a positive Murphy's sign. Ultrasonographic examination showed a hydropic gallbladder without stones and he underwent a cholecystectomy. Pathological examination revealed an intramural nodule (9 mm) in the neck region which is consisted of acini, ducts and islet cells of an aberrant pancreatic tissue. Although HP is encountered rarely in the gallbladder and is found incidentally during pathological studies, this case emphasizes that HP might cause symptoms and present clinically as cholecystitis. For this reason, in patients presenting with symptomatic gallbladder diseases, including cholecystitis without any other pathology, HP should be taken into consideration before it is diagnosed as "idiopathic".

Advanced-stage gastrointestinal stromal tumor treated with imatinib in a 12-year-old girl with a unique mutation of PDGFRA

A 12-year-old girl presented with a large abdominal tumor. At surgery, a huge pedunculated extraluminal tumor was found arising from the greater curvature of the stomach and invading the surrounding structures, and there were also a submucosal tumor measuring 5 × 4 × 4 cm and multiple intramural nodules beside the main tumor. These lesions, which were removed with 1.0-cm surgical margins, were immunohistochemically positive for c-kit (CD117) and CD34. A diagnosis of gastrointestinal stromal tumor (GIST) was made. The huge size of the tumor (3.6 kg in weight and 36 × 25 × 25 cm in diameter), the invasion of the surrounding structures, and the increased mitotic figures indicated the GIST had malignant potential. Sequence analysis of the polymerase chain reaction product of RNAs from the tumor cells revealed a novel platelet-derived growth factor receptor α ( PDGFRA) mutation, which would exhibit biologic consequences similar to those of the c-kit mutation. The patient underwent a 3-month course of imatinib mesylate as adjuvant chemotherapy because of the possible risk for tumor recurrence. She is now doing well without any evidence of recurrence or metastasis 25 months after the surgery. Only 9 cases of GIST have been reported in children, and a review of those cases revealed GISTs in children would be associated with a better prognosis than in adults and that one third of pediatric GISTs presented with intestinal obstruction in the newborn period.

Comparison of intramural myomectomy scar after laparotomy or laparoscopy

Optimal uterine repair after myomectomy is important to avoid menstrual disturbances and allow for successful subsequent pregnancies. Apart from patients’ individual characteristics, different surgical approaches might be accountable for different healing processes. The objective of this study was to compare the morphology of scars resulting from laparoscopic and laparotomic myomectomy. Fifteen patients (age range, 22–35 years) who underwent cesarean delivery because of a previous myomectomy were included. In all patients myomectomy had been performed for a single intramural nodule located in the anterior, posterior, or fundal uterine wall. The surgical approach was either laparotomic myomectomy (ns10) or laparoscopic myomectomy (ns5). The postoperative course was uncomplicated in all patients. The laparotomic myomectomies were performed by the same surgeon whereas the laparoscopic myomectomies were performed by two different surgeons. The surgical technique for optimal uterine repair following laparotomic myomectomy was similar to that previously described for myomectomy dur-

Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases.

In this study we analyzed the clinicopathologic features of duodenal smooth muscle or stromal tumors, including 156 GISTs, 6 leiomyomas (LMs), and 5 leiomyosarcomas (LMSs) from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki. GISTs were documented as KIT positive (n = 109); 47 tumors were also included because of their histologic identity to KIT-positive cases. GIST-specific c-kit gene mutations were documented in exon 11 in 9 of 30 cases (30%) and exon 9 in 4 of 30 cases (13%). The GISTs occurred in patients with an age range of 10-88 years (median 56 years); 54% were male. Ten patients had neurofibromatosis type I; six of them had multiple GISTs. The GISTs ranged from small asymptomatic intramural or external nodules to large masses that extended into the retroperitoneum (median size 4.5 cm). They were mostly spindle cell tumors; three malignant GISTs had an epithelioid morphology, and 81 cases had skeinoid fibers. The tumors often coexpressed CD34 and KIT (54%) and were variably positive for smooth muscle actin (39%) and S-100 protein (20%) but never for desmin. A total of 86% of patients with tumors >5 cm with >5 mitoses/50 high power fields (HPF) (n = 21) died of disease, whereas no tumor <2 cm with <5 mitoses/50 HPF (n = 12) recurred or caused death. Long latency was common between primary operation and recurrences or metastases; either one occurred in 49 of 140 patients with follow-up (35%). No formula could accurately predict metastases, which occasionally developed even if mitotic activity was <5/50 HPF and size <5 cm. Metastases were in the abdominal cavity, liver, and rarely in bones and lungs but never in lymph nodes. Four actin- and desmin-positive and KIT-negative benign intramural LMs were similar to those more often seen in the esophagus. There were five LMSs, one of which formed a polypoid intraluminal mass; all were actin positive and KIT negative. The great majority of duodenal mesenchymal tumors are GISTs, which have a spectrum from small indolent tumors to overt sarcomas. LMs and LMSs are rare.

Multidetector CT of high-risk patients with occult gastrointestinal stromal tumors.

Stromal tumors are an important cause of occult gastrointestinal bleeding in patients who are middle aged or older. This study evaluated the size at which stromal tumors become symptomatic and necrotic and examined the role and limitations of multidetector CT in detecting gastrointestinal stromal tumors. Two hundred seventy-one patients with stromal tumors were retrospectively studied to examine the size of symptomatic, necrotic, or malignant stromal tumors. Next, five asymptomatic family members with germline c-kit mutations were prospectively screened to evaluate the role and limitations of multidetector CT. In this retrospective study, two thirds of the patients with stromal tumors had symptoms and signs that were correlated with tumor size. Stromal tumors larger than 3 cm were accompanied by necrosis, whereas this finding was rare for those smaller than 3 cm. In the prospective screening, multidetector CT with IV infusion of contrast material showed gastrointestinal stromal tumors of more than 2 cm as slightly enhanced intramural or extramural nodules and also detected most gastrointestinal stromal tumors between 1 and 2 cm. Stromal tumors of less than 3 cm were not accompanied by necrosis and appeared as slightly enhanced homogenous tumors with the administration of contrast media. Screening with multidetector CT should be feasible for detecting symptomatic stromal tumors of unknown location in the gastrointestinal tract.

Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the rectum and anus: a clinicopathologic, immunohistochemical, and molecular genetic study of 144 cases.

Gastrointestinal stromal tumors (GISTs), the specific KIT-positive mesenchymal tumors of the gastrointestinal tract, have been sporadically reported in the rectum, but there are few clinicopathologic series. In this study we analyzed the clinicopathologic features of 133 anorectal GISTs, 3 intramural leiomyomas (LMs), and 8 leiomyosarcomas (LMSs) from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki. Ninety-six GISTs were documented as KIT-positive and three additional ones as CD34-positive. Thirty-four tumors were included by their histologic similarity to KIT- or CD34-positive cases. GIST-specific c-kit gene mutations, mostly in exon 11, were documented in 18 of 29 cases (62%). The GISTs occurred in adults with the age range of 17-90 years (median 60 years) with a significant male predominance (71%). The tumors ranged from small asymptomatic intramural nodules to large masses that bulged into pelvis causing pain, rectal bleeding, or obstruction. They were mostly highly cellular spindle cell tumors; four tumors had an epithelioid morphology. The tumors coexpressed CD34 and KIT and were rarely positive for smooth muscle actin or desmin and never for S-100 protein. Seventy percent of patients with tumors >5 cm with more than 5 mitoses/50 high power fields (HPF) (n = 31) died of disease, whereas only one tumor <2 cm with <5 mitoses/50 HPF (n = 21) recurred and none caused death. Long latency was common between primary operation and recurrences and metastases; either one occurred in 60 of 111 patients with follow-up (54%). Distant metastases were in the liver, bones, and lungs. Three benign actin- and desmin-positive and KIT-negative intramural LMs, similar to those seen in the esophagus, were identified. There were eight LMSs, six of which formed a polypoid intraluminal mass and were actin-positive and KIT-negative. Despite high mitotic counts, only one LMS patient died of disease. A great majority of rectal smooth muscle and stromal tumors are GISTs, which have a spectrum from minimal indolent tumors to overt sarcomas. Intramural LMs are exceptional, and true LMSs are rare, and similar to colonic ones, often present as intraluminal polypoid masses that appear to have a better prognosis than GISTs with similar mitotic rates.

Intramural Hypoattenuated Nodules in Thickened Wall of the Gallbladder: CT Features According to Their Primary Causes

According to published reports, a common feature of xanthogranulomatous chole-cystitis is the presence of intramural hypoattenuated nodules in thickened gallbladder wall. These nodules can, however, also be seen in pathological conditions such as acute cholecystitis, hyperplastic cholecystoses (cholesterolosis and adenomyomatosis), gallbladder cancer, and other inflammatory diseases such as tuberculosis. Retrospective review of the abdominal CT findings in 622 patients who for various reasons underwent cholecystectomy during a one-year period showed that intramural nodules were present in 60. In this pictorial essay we illustrate the imaging features of the many different pathological conditions which give rise to intramural hypoattenuated nodules in thickened wall of the gallbladder, correlating these features with the histopathological findings.

Uterine Metastasis from a Heterologous Metaplastic Breast Carcinoma Simulating a Primary Uterine Malignancy

Objective. To describe the first distant metastasis of a heterologous metaplastic breast carcinoma in the uterus and discuss its differential diagnosis.Methods. Light microscopy, immunohistochemistry, and flow cytometry were used to evaluate the tumor.Results. A 58-year-old woman underwent mastectomy for metaplastic breast carcinoma confined to the breast. She presented 4 years later with vaginal bleeding. The endometrial curettage showed a poorly differentiated carcinoma. She underwent hysterectomy and bilateral salpingo-oophorectomy as well as pelvic and periaortic lymphadenectomy. Clinical and intraoperative findings favored a primary uterine malignancy. The uterus was markedly distorted with multiple gray-white, solid subserosal, and intramural tumor nodules. The tumor diffusely infiltrated the endometrium sparing benign endometrial glands. The tumor nodules were distributed full thickness of the myometrium. These nodules were composed of high-grade malignant epithelial cells with areas of chondroid metaplasia. Extrauterine microscopic tumor was present in left ovary, pelvic, and periaortic lymph nodes. The histologic features and estrogen/progesterone receptors (ER/PR) as well as DNA ploidy analysis of the uterine tumor showed striking similarity with those of the primary metaplastic breast carcinoma. A diagnosis of metastatic metaplastic breast carcinoma in the uterus was rendered.Conclusion. A metastatic heterologous metaplastic breast carcinoma with cartilaginous metaplasia should be considered in the differential diagnosis of heterologous uterine malignant mixed mesodermal tumor (MMMT) and high-grade endometrioid carcinoma with rare foci of cartilage.

Xanthogranulomatous cholecystitis: radiologic findings with histologic correlation that focuses on intramural nodules.

The purpose of this study was to histologically classify intramural nodules associated with xanthogranulomatous cholecystitis and to evaluate the radiologic findings for each type of nodule. Pathologic slides and radiologic studies including 14 sonographic and 16 CT examinations in 19 patients (12 men, seven women; mean age, 61 years) with xanthogranulomatous cholecystitis were reviewed. Radiologic findings were correlated with the histologic type of intramural nodule: abscess, xanthogranuloma, or a combination of the two. The duration of symptoms for each type of intramural nodule was also evaluated. Histologically, all patients had intramural nodules that were either abscesses (n = 11), xanthogranulomas (n = 5), or a combination of the two (n = 3). Radiologic studies revealed nodules in 10 patients (52.6%; four abscesses, four xanthogranulomas, and two combinations). For abscesses, the mean interval from onset of symptoms to surgery was 25 days; for xanthogranulomas, 70 days (p = .0057). Abscesses were associated with more complications of xanthogranulomatous cholecystitis. Intramural nodules in patients with xanthogranulomatous cholecystitis were found to represent abscesses or xanthogranulomas at histology. Xanthogranulomas were more often revealed radiologically than were abscesses. Abscesses caused more clinical complications. Because symptoms lasted longer for xanthogranulomas, we hypothesized that abscesses may become xanthogranulomas.

US findings of xanthogranulomatous cholecystitis

The ultrasound (US) scans of eleven patients with histologically proven xanthogranulomatous cholecystitis have been reviewed. The thickness of the gallbladder (GB) wall, echogenicity of the thickened GB wall, the presence of intramural nodules, gallstones, pericholecystic fluid, loss of wall definition with the liver, and intrahepatic ductal dilatation were specifically assessed by two radiologists. The range of thickness of the GB wall was 3-20mm (mean, 11.2 mm). Thickening was diffuse in nine cases and focal in two. The thickened GB wall, excluding intramural nodules, was echogenic in seven, isoechoic in three and hypoechoic in one, compared with hepatic echogenicity. Intramural nodules were found in eight cases. These were discrete, oval or flat, and of low echogenicity. These ranged in size from 6 to 12 mm (mean, 10.5 mm). Gallstones were present in six cases and there was intrahepatic duct dilatation in two of these. Definition between the liver and the GB was obliterated in seven cases. One case of GB perforation, confirmed at operation, demonstrated pre-operatively a GB wall defect and connection with the surrounding pericholecystic fluid. Pericholecystic fluid was absent in the other 10 cases. Xanthogranulomatous cholecystitis presents ultrasonically as GB wall thickening, and the majority of cases in this series also demonstrated intramural hypoechoic nodules. These findings can be helpful in the diagnosis of xanthogranulomatous cholecystitis.

I foci iperintensi in corso di neurofibromatosi hanno sempre una evoluzione benigna?

More than 70% of patients with neurofibromatosis type 1 (NF-1), present hyperintense foci in MR DP-T2 weighted images, located in deep grey and white matter. The true nature of these hyperintense foci is still unknown: they could be hamartomas, that is composition and organisation alterated areas of cytologic normal tissue, gliotic areas, delayed or disorganised areas of myelinisation and, finally, low grade glial tumors (astrocytoma). We present the case of a 17 years old pazient, who underwent 8 MR brain studies in a six years period. We analyzed the MR characteristics of the hyperintense foci in seriated studies and, particularly, the evolution of a single, simple, hyperintense periventricular lesion in cistic tumor with intramural nodule.

Xanthogranulomatous cholecystitis: CT features with emphasis on differentiation from gallbladder carcinoma.

To evaluate the computed tomographic (CT) features of xanthogranulomatous cholecystitis (XGC) and to distinguish it from gallbladder carcinoma. Retrospective analysis was performed in 11 patients with XGC and 17 patients with gallbladder carcinoma in which the wall was thickened. The following CT features were analyzed: maximum wall thickness, intramural hypoattenuated nodules, mucosal line, patterns of wall thickening and enhancement, and the presence of stones. The changes outside the gallbladder were also compared. The mean thickness of the gallbladder wall was 1.8 cm in patients with XGC and 2.1 cm in patients with gallbladder carcinoma. Intramural hypoattenuated nodules were seen in all patients with XGC but in only seven patients with gallbladder carcinoma (P = .008). The mucosal line was observed in nine patients with XGC and in six with gallbladder carcinoma (P = .02). The gallbladder wall was more diffusely thickened in patients with XGC (10 of 11 patients) than in patients with gallbladder carcinoma (seven of 17 patients) (P = .01). The occurrence of changes outside the gallbladder did not differ statistically significantly. Because of a statistically significant overlap of CT features, only when intramural hypoattenuated nodules occupy a large area of the thickened gallbladder wall can the diagnosis of XGC be highly suggestive. The diagnosis of XGC at CT may indicate a less aggressive surgical approach.

Neuromesenchymal Hamartoma of the Small Bowel

We present the case of a long small bowel stricture with pathological features similar to those described as "neuromuscular and vascular hamartoma," showing in addition extensive fatty submucosal infiltration and fibrous intramural nodules. In the controversy about the nature of this disorder, we consider it a separate entity. Taking into account that other mesenchymal tissues than the originally described can participate, we propose the alternative term of "neuromesenchymal hamartoma" of the small bowel.

Carcinoid tumors of the alimentary tract. I. Radiographic diagnosis.

This paper is based on a retrospective analysis of the radiographic findings in 18 new proven cases of carcinoids of the gastrointestinal tract. Examples of lesions involving different segments together with a detailed review of the pertinent literature are provided. Background information regarding the malignant potential of all carcinoids with particular reference to invasiveness, size, site, and symptomatology is discussed. Depending on the time of the examination during their development, carcinoids present either as smoothly defined, mostly small intramural nodules, or as large infiltrating and ulcerating malignant looking tumors. When mesenteric infiltration is present, a more specific combination of findings composed of submucosal nodules, separation of loops, and localized kinking may be seen. The demonstration of an isolated, sharply contoured defect does not exclude malignancy. The less common radiographic manifestations of tumoral calcifications and bony metastases are reviewed and illustrated.