Predictors of Malignancy in “Pure” Branch-Duct Intraductal Papillary Mucinous Neoplasm of the Pancreas without Enhancing Mural Nodules on CT Imaging: A Nationwide Multicenter Study

BACKGROUND/AIMS: Presence of enhanced mural nodules, which can be visualized using computed tomography (CT), is one of high-risk stigmata in branch-duct intraductal papillary mucinous neoplasms (BD-IPMNs). Conversely, the absence of enhanced mural nodules on preoperative imaging does not exclude malignant risk. The present study aimed to investigate other morphological features as predictors of malignancy in “pure” BD-IPMNs without enhanced mural nodules on CT. METHODS: This retrospective study included 180 patients with surgically confirmed “pure” BD-IPMNs of the pancreas and no enhanced mural nodules on preoperative CT. The study was conducted at 15 tertiary referral centers throughout South Korea. Univariate and multivariate analyses were used to identify significant predictors of malignancy. RESULTS: BD-IPMNs with low-grade (n=84) or moderate-grade (n=76) dysplasia were classified as benign; those with high-grade dysplasia (n=8) or invasive carcinoma (n=12) were classified as malignant. The multivariate analysis revealed that cyst size ≥30 mm (odds ratio, 8.6; p=0.001) and main pancreatic duct diameter ≥5 mm (odds ratio, 4.1; p=0.01) were independent risk factors for malignancy in “pure” BD-IPMNs without enhanced mural nodules on CT. Endoscopic ultrasound detected enhanced mural nodules (6/82) that had been missed on CT, and two IPMNs with enhanced mural nodules were malignant. CONCLUSIONS: In patients with “pure” BD-IPMNs who have no enhanced mural nodules on CT, cyst size ≥30 mm and main pancreatic duct diameter ≥5 mm may be associated with malignancy.

Determination of Malignant and Invasive Predictors in Branch Duct Type Intraductal Papillary Mucinous Neoplasms of the Pancreas: A Suggested Scoring Formula

Prediction of malignancy or invasiveness of branch duct type intraductal papillary mucinous neoplasm (Br-IPMN) is difficult, and proper treatment strategy has not been well established. The authors investigated the characteristics of Br-IPMN and explored its malignancy or invasiveness predicting factors to suggest a scoring formula for predicting pathologic results. From 1994 to 2008, 237 patients who were diagnosed as Br-IPMN at 11 tertiary referral centers in Korea were retrospectively reviewed. The patients' mean age was 63.1 +/- 9.2 yr. One hundred ninty-eight (83.5%) patients had nonmalignant IPMN (81 adenoma, 117 borderline atypia), and 39 (16.5%) had malignant IPMN (13 carcinoma in situ, 26 invasive carcinoma). Cyst size and mural nodule were malignancy determining factors by multivariate analysis. Elevated CEA, cyst size and mural nodule were factors determining invasiveness by multivariate analysis. Using the regression coefficient for significant predictors on multivariate analysis, we constructed a malignancy-predicting scoring formula: 22.4 (mural nodule [0 or 1]) + 0.5 (cyst size [mm]). In invasive IPMN, the formula was expressed as invasiveness-predicting score = 36.6 (mural nodule [0 or 1]) + 32.2 (elevated serum CEA [0 or 1]) + 0.6 (cyst size [mm]). Here we present a scoring formula for prediction of malignancy or invasiveness of Br-IPMN which can be used to determine a proper treatment strategy.

Determination of Malignancy Predictors in Branch Duct TypeIntraductal Papillary Mucinous Neoplasms of the Pancreas

PURPOSE: Branch duct type intraductal papillary mucinous neoplasms (Br-IPMN) have better prognosis and lower malignancy rate than do main duct type IPMNs (M-IPMN). However, surgical resection is necessary when malignancy is suspected, and radical resection should be performed in cases of invasive IPMN. The objectives of this study were to investigate the characteristics of Br-IPMN and related predictive malignancy/invasiveness factors and to present a standardized scoring system for predicting pathologic results. METHODS: We conducted a retrospective review of 91 patients diagnosed with Br-IPMNs between 1998 and 2007. Mean patient age was 62.5+/-9.0 years. Eighty (87.9%) patients had benign IPMNs (17 adenoma, 63 borderline malignancy), and 11 (12.1%) patients had malignant IPMNs (4 carcinoma in situ, 7 invasive carcinoma). Eighty-four (92.3%) patients had noninvasive IPMN, and 7 (7.7%) patients had invasive IPMN. RESULTS: The size of the cystic mass and the presence and size of the mural nodule were found by univariate analysis to be significantly different between the benign and malignant groups. History of DM, size of the cystic mass, size of the mural nodule, and thickness of the cystic wall were significantly different between the noninvasive and invasive IPMN groups on univariate analysis. The regression coefficients for the size of the cystic mass and for the size and presence of the mural nodule were calculated using multivariate analysis, and the scores predicting malignant and invasive IPMN determined (P=0.001, P=0.000, respectively). CONCLUSION: There are no universally held guidelines for surgical management of Br-IPMN patients. Hence, many clinicians have difficulty deciding the specific character of the resection they will pursue. By using malignancy- and invasiveness-predicting scores, we expect that much of this difficulty can be avoided in the future.

Pleomorphic Xanthoastrocytoma with an Intracystic Hemorrhage: A Case Report and Literature Review

Pleomorphic xanthoastrocytoma (PXA) has been considered as a low grade tumor of adolescents and young adults. Although this tumor often shows cystic component, the hemorrhage within the cyst is extremely rare. The authors report a rare case of cystic PXA with a hemorrhage within the cyst and the mural nodule in the left frontal lobe. A 64-year-old male presented with a week history of the right side hemiparesis. After gross total resection of the tumor, the patient was fully recovered from neurological deficit. It is suggested that this typically benign tumor could be presented with hemorrhage, causing a rapid neurological deterioration. Prompt surgical intervention, especially total removal of the tumor can provide an excellent functional recovery.

A Case of Mucinous Cystadenoma with Sarcoma-Like Mural Nodule / 대한산부인과학회잡지

Mural nodules in mucinous ovarian tumors are very rare. Since Prat and Scully first described sarcomatous and sarcoma-like mural nodules within mucinous ovarian tumors in 1979, only about 30 cases have been reported in the literature. Pathologically, mural nodules have three different types; i.e. 1) sarcoma-like mural nodule, 2) sarcomatous mural nodule, 3) anaplastic carcinomatous mural nodule. It is important to distinguish sarcoma-like mural nodule from another two type. because the former in the mucinous ovarian tumor has more favorable prognosis than the latter. We report a case of mucinous cystadenoma with sarcoma-like mural nodules in a 23-year-old nulliparous woman.

Ovarian Mucinous Cystadenocarcinoma with Mural Nodules of Anaplastic Carcinoma: A case report

A case of an ovarian mucinous cystadenocarcinoma with mural nodules of anaplastic carcinoma is reported. The patient was a 46-year-old female with a right ovarian cystic mass and underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. The patient died of the disease in five months. Macroscopically, the right ovarian cyst wall contained numerous well-demarcated mural nodules. Histologically, the cysts were mucinous cystadenocarcinoma, and the nodules were composed of sarcoma-like spindle and polygonal cells with atypia and numerous mitoses. Spindle cells in the mural nodule showed epithelial characteristics by electron microscopy and immunohistochemistry. This case supports an epithelial origin of the mural nodule of anaplastic carcinoma in ovarian mucinous tumors.

Sarcoma-like Mural Nodule in Ovarian Mucinous Cystadenocarcinoma: A case report

We describe an ovarian mucinous cystadenocarcinoma with a sarcoma-like mural nodule. In the literature, rare cases of ovarian mucinous tumors have been described which contain foci of undifferentiated carcinoma, sarcoma, and sarcoma-like nodules. The distinction between these lesions is important because of poorer prognosis of true sarcoma and anaplastic carcinoma than sarcoma-like mural nodules. This case shows different results of immunohistochemical stain for anaplastic carcinoma.

A Case of Ovarian Mucinous Cystadenocarcinoma with Mural Nodule of Anaplastic Carcinoma / 대한부인종양콜포스코피학회잡지

Mural nodules in ovarian mucinous tumors, whether benign, borderline, or malignant, have been described by several authors since Prat and Scully first described sarcoma or sarcoma-like mural nodules occurring in association with mucinous epithelial neoplasia in 1979. Three distinct types of the mural nodule have been identified; i. e., 1) sarcoma-like lesion, 2) true mesenchymal sarcome, and 3) anaplastic carcinoma simulating a sarcoma. Mural nodules of sarcomatous and carcinomatous foci associated with mucinous ovarian tumors should be separated from sarcoma-like nodules because of the poor prognosis of the former compared to the favorable prognosis of the latter. We experienced a case of ovarian mucinous cystadenocarcinoma with sarcoma-appearing mural nodule of anaplastic carcinoma occurred in a 59-year-old woman and reported with brief review of the literatures.

Posterior Fossa Hemangioblastoma

Hemangioblastomas are histologically benign tumors, which generally occur at the posteriro fossa and rarely in supratentorial region. The authors analyzed 18 cases of posterior fossa hemangioblastoma which have been operated at the Department of Neurosurgery, Seoul National University Hospital from 1982 to 1989. The clinico-pathological and radiological features were as follows : 1) Among 18 cases, 13 cases were cystic type with or without mural nodule and 5 cases were solid type. 2) 2 cases were compatible with Von Hippel Lidau's complex, one of which was associated with retinal angiomatosis and the other with mulitple cysts in the pancreas and kidney. In 3 cases, hemoglobin level was above 18g/dl, implying polycythemia, but returned to normal level postoperatively. 3) Mural nodules were not visible in the enhanced CT scan in the 2 cases, in one of which mural nodule was visible in the angiography and so, angiography was more helpful than the CT scan in the detection and the localization of the mural nodule. 4) Among 18 cases, 2 cases(11%) recurred at 34 months and 48 months postperatively.

A Case of Primitive Clioma

Among the intracranial glioma, primitive glioma is very rare. We report a case of primitive glioma involving left parietofrontotemporal lobe of 8-year-old boy who had about 12 months duration of right sided hemiparesis, headache, vomiting and papilledema. CT scan showed a large, well defined round area of low density with peripheral rim of high density and high density small mass suggesting mural nodule in left frontoparietotemporal region. A left frontoparietotemporal osteoplastic craniotomy was carried out. A small nodule and cystic membrane containing pale yellowish colored proteinous cystic fluid were almost totally removed without difficulty. Postoperatively, the right hemiparesis and headache gradually cleared. Pathologic diagnosis was primitive glioma.