Macrocystic Tumors Research Articles

Presentation, imaging, patterns of care, growth, and outcome in sporadic and von Hippel-Lindau-associated central nervous system hemangioblastomas.

Hemangioblastoma is a relatively rare neoplasm occurring mostly in the cerebellum that may arise sporadically or in the context of von Hippel-Lindau (VHL) syndrome. Presentation, imaging, natural history, surgical patterns of care, and outcomes are incompletely defined for this uncommon lesion. We reviewed our large institutional series to help clarify these issues. Retrospective analysis of consecutive, neurosurgically managed CNS hemangioblastomas at Mayo Clinic, 1988-2018. Two hundred and eighty five hemangioblastomas were treated in 184 unique patients (115 sporadic, 69 VHL). Compared to sporadic patients, VHL patients were younger (36.7 vs 51.7years; p < 0.0001), were treated while asymptomatic more commonly (47.3 vs 4.2%; p < 0.0001), had smaller lesions (6.6 vs 13.9mL; p < 0.0001), and harbored lesions with associated cysts less frequently (51.0 vs 75.0%; p = 0.0002). Macrocystic tumor architecture was associated with larger lesion size and greater symptom severity. Solid lesions later formed cysts at a median 130months. Growth in both total volume and solid component accelerated after cyst formation (10.6 and 6.0 times median rate prior to cyst emergence). VHL patients died at a younger age (47.9 vs 74.5, p = 0.0017) and were more likely to die of direct disease sequelae. Though treatment-free survival time was significantly longer in sporadic cases, a substantial fraction (> 40%) developed tumor recurrence/progression requiring additional treatment. Hemangioblastoma presentation varies with etiology and clinical course is more complicated in VHL cases. Nodular lesions often develop cysts over time which is associated with accelerated tumor growth. Sporadic cases have a previously unappreciated but substantial risk of late recurrence/progression requiring treatment.

Clinicoradiological features of resected serous cystic neoplasms according to morphological subtype and preoperative tentative diagnosis: can radiological characteristics distinguish serous cystic neoplasms from other lesions?

PurposeSerous cystic neoplasm (SCN) of the pancreas is considered benign in most cases. However, some SCN patients undergo surgical resection because lesions could not be differentiated preoperatively. This study evaluated causes of resection for SCN, investigated clinical and radiological features of surgically resected SCNs, and compared characteristics of SCNs diagnosed accurately and those misdiagnosed.MethodsOne hundred patients, who underwent surgery for pancreatic cystic tumors with pathological confirmation of SCN between 2000 and 2014 were retrospectively analyzed.ResultsThe mean patient age was 52.9 years, 67 (67%) were female, and most lesions (72%) were located in the pancreatic body or tail. Fifty-one (51%) pathologically confirmed SCNs were preoperatively diagnosed as non-SCNs. Patients underwent surgery due to uncertain diagnosis (58%) or symptomatology (18%). According to radiological examination, most lesions were macrocystic (85%), exhibited septation (58%), or were enhancing lesions (48%). Compared with preoperatively diagnosed non-SCNs, accurately diagnosed SCNs exhibited septation (75.5% vs. 41.2%, P = 0.001) and central scar (36.7% vs. 11.8%, P = 0.003) more frequently in radiological examinations. In terms of macrocystic tumors (n = 85), most parameters did not differentiate preoperative diagnoses, although lesions accurately diagnosed as SCN exhibited septation more frequently than those preoperatively misdiagnosed as mucinous cystic neoplasm or intraductal papillary mucinous neoplasm (70.7% vs. 38.9% vs. 33.3%, respectively, P = 0.009).ConclusionIt is difficult to accurately distinguish macrocystic SCNs from other cystic tumors using conventional radiological methods. For more accurate diagnosis, new biomarkers and/or other diagnostic modalities are needed and warrant further investigation.

Incidence and reasons of pancreatic resection in patients with asymptomatic serous cystadenoma

Background/ObjectivesDespite diagnostic refinements, pancreatic resection (PR) is eventually performed in some patients with asymptomatic serous cystadenoma (A-SCA). The aim of this study was to define incidence and reasons of PR in A-SCA. MethodsA retrospective analysis of a prospectively maintained database was performed for all the patients referred for pancreatic cystic lesions (PCL) between January 2005 and March 2016. ResultsOverall, there were 1488 patients with PCL, including 1271 (85.4%) with incidental PCL (I-PCL). During the study period referral of I-PCL increased 8.5-fold. Surgery was immediately advised in 94 I-PCL (7.3%) and became necessary later on in 11 additional patients (0.9%), because of the development of symptoms. Overall, PR was performed in 105/1271 patients presenting with I-PCL (8.2%), including 27 with A-SCA (2.1%). All patients with A-SCA underwent ultrasonography and contrast-enhanced computed tomography. Magnetic resonance imaging was performed in 21 patients (77.8%), 18 F-FDG positron emission tomography in 8 (29.6%), endoscopic ultrasonography (EUS) in 2 (7.4%), and EUS-guided fine needle aspiration (EUS-FNA) in 1 (3.7%). These studies demonstrated a combination of atypical features such as solid tumor (3; 11.1%), oligo-/macrocystic tumor (24; 88.8%), mural nodules (14; 51.8%), enhancing cyst walls (17; 62.9%), dilation of the main pancreatic duct (3; 11.1%), and upstream pancreatic atrophy (1; 3.7%). Additionally, 14/27 patients (51.8%) were females with oligo-/macrocystic tumors located in the body-tail of the pancreas. ConclusionsManagement of patients with A-SCA entails a small risk of PR especially when these tumors demonstrate atypical radiologic features associated with confounding anatomic and demographic characteristics.

Cystic Vestibular Schwannomas Respond Best to Radiosurgery.

Vestibular schwannomas (VS) have a well-documented response to Gamma Knife® (Elekta AB, Stockholm, Sweden) Stereotactic radiosurgery (SRS). However, there are limited data available regarding the volumetric response of cystic tumors. This report correlates the radiographic appearance of VS before radiosurgery with the delayed volumetric response. This study reviewed our SRS experience with 219 VS patients between 2003 and 2013. Patients were treatment naïve and had a significant extracanalicular tumor volume. Magnetic resonance imaging at the time of SRS identified 42 contrast-enhancing macrocystic tumors, 45 contrast-enhancing microcystic tumors, and 132 homogeneously enhancing tumors with no intratumoral cyst formation. The median follow-up was 49.1 months. The median tumor volume was 2.6 cm 3 (0.70-16.1 cm 3 ) and the median dose was 12.5 Gy (11-13 Gy). The actuarial tumor control rate was 99.4% at 2 years and 96.4% at 5 years. A volumetric reduction of >20% occurred in 85.4% of macrocystic tumors, 76.1% of microcystic tumors, and 62.8% of homogeneously enhancing VS. The median volume decrease per year for macrocystic, microcystic, and homogenous tumors was 17.2%, 7.5%, and 7.9% per year respectively ( P < .001). A 2:1 blinded volumetric case match showed a significant size reduction in macrocystic tumors compared to noncystic tumors ( P = .007). Serviceable hearing was maintained in 61.5% of patients that had Gardner-Robertson grade I-II hearing before treatment. Surgical resection or repeat radiosurgery was performed in 8 patients (3.6%) who had sustained tumor progression. SRS provided VS tumor control in >95% of patients, regardless of radiographic characteristics. Tumor volume regression was most evident in patients with cystic tumors.

C.01 Cystic Vestibular Schwannomas respond best to radiosurgery

Background: Vestibular Schwannomas (VS) have a well- documented response to Gamma Knife® Stereotactic radiosurgery (SRS). However, there is limited data available regarding the volumetric response of cystic tumors. This report correlates the radiographic appearance of VS before radiosurgery with the delayed volumetric response. Methods: This study reviewed 219 VS patients between 2003 and 2013. Patients were treatment naïve and had a significant extracanalicular tumor volume. MRI at SRS identified; 42 contrast enhancing macrocystic tumors, 45 contrast enhancing microcystic tumors, and 132 homogeneously enhancing tumors with no intra-tumoral cyst formation. The median follow-up was 49.1 months. The median tumor volume was 2.6cm3 (0.70-16.1cm3) and the median dose was 12.5Gy (11-13Gy). Results: The actuarial tumor control rate was 99.4% at 2-years and 96.4% at 5-years. A volumetric reduction of &gt;20% occurred in 85.4% of macrocystic tumors, 76.1% of microcystic tumors and 62.8% of homogeneously enhancing VS. The median volume decrease per year for macrocystic, microcystic and homogenous tumors was 17.2%, 7.5% and 7.9% per year respectively (p&lt;0.001). Serviceable hearing was maintained in 61.5% of patients that had Gardner-Robertson grade I-II hearing. Conclusions: SRS provided VS tumor control in &gt;95% of patients, regardless of radiographic characteristics. Tumor volume regression was most evident in patients with cystic tumors.

Value and Limitations of Diffusion-Weighted Imaging in Grading and Diagnosis of Pediatric Posterior Fossa Tumors: Fig 1.

DWI reportedly accurately differentiates pediatric posterior fossa tumors, but anecdotal experience suggests limitations. In 3 years, medulloblastoma and JPA were differentiated by DWI alone in 23/26 cases (88%). Ependymoma (n = 5) could not be reliably differentiated from medulloblastoma or JPA. A trend toward increased diffusion restriction in higher grade tumors (1/14 grade I, 7%; 9/12 grade IV, 75%) had too much overlap to predict the grade of individual cases. The overlap in ADC between tumor types appeared partly due to technical factors (in small, heterogeneous, calcific, or hemorrhagic tumors) but also likely reflected true histologic variability, given that our 3 overlap cases included a desmoplastic medulloblastoma, an anaplastic ependymoma, and a JPA with restricted diffusion in its nodule. Simple structural features (macrocystic tumor, location off midline) aided in distinguishing JPA from the other tumors in these cases.

Prolactin–Growth Factor Crosstalk Reduces Mammary Estrogen Responsiveness Despite Elevated ERα Expression

Most breast cancers that occur in women express estrogen receptor alpha (ERalpha). However, a large subset of these cancers either does not initially respond to anti-estrogen therapy or develops resistance to such treatment modalities. One postulated mechanism of this failure is signaling cross talk between hormones and local growth factors. To examine these complex interactions in vivo, we assessed the effects of estrogen on transforming growth factor alpha (TGFalpha)- and prolactin (PRL)-induced mammary tumorigenesis in transgenic mice. Both PRL and estrogen reduced the latency of TGFalpha-induced oncogenesis, resulting in tumors that were variably ERalpha-positive, but were progesterone receptor-negative. However, despite elevated ERalpha levels in NRL-PRL/TGFalpha glands, tumor latency was not reduced with increasing estrogen levels, nor increased after ovariectomy. Furthermore, PRL and TGFalpha in combination blocked the mitogenic effects of estrogen, dramatically reduced progesterone receptor levels, and diminished ERalpha down-regulation in response to circulating estrogen levels, in contrast to the other genotypes. Notably, however, ductal morphology remained responsive to estrogen, indicating that TGFalpha and PRL in combination can inhibit some, but not all, estrogenic signals. Both in vitro and in vivo, PRL and TGFalpha cooperatively enhanced Akt phosphorylation, which is associated with endocrine resistance in human disease. These findings provide insight into the interactions of PRL with growth factors during mammary oncogenesis and suggest combinatorial approaches that may result in improved therapeutic efficacy.

Magnetic Resonance Imaging of Cystic and Endocrine Pancreatic Neoplasms

This article describes the appearance of miscellaneous cystic and endocrine neoplasms using magnetic resonance imaging (MRI). Magnetic resonance imaging is a useful diagnostic modality in the assessment of various pancreatic neoplasms. Pancreatic endocrine tumors are moderately low in signal intensity on T1-weighted fat-suppressed images and moderately high in signal intensity on T2-weighted fat-suppressed images and demonstrate homogeneous, ring, or diffuse heterogeneous enhancement on immediate postgadolinium gradient echo images. Cystic pancreatic neoplasms, including intraductal papillary mucinous neoplasm, are well demonstrated and subcategorized according to their characteristic cystic configurations on MRI and MR cholangiopancreatography images. Microcystic serous cystadenomas are demonstrated as a honeycombed appearance on T2-weighted images and have a central stellate scar. Mucinous cystic neoplasms usually appear as multilocular cystic masses, with benign forms of macrocystic tumors possessing uniform thickness septations and malignant forms exhibiting irregular septations and tumor nodules. The presence of tumor stroma, invasion of adjacent tissue, or liver metastases can be assessed by MRI. The connection between the pancreatic duct and the cystic tumor is usually well shown on MR cholangiopancreatography images.

The Morphological Classification of a Serous Cystic Tumor (SCT) of the Pancreas and Evaluation of the Preoperative Diagnostic Accuracy of Computed Tomography

Although the honeycomb microcystic type is common and typical for a serous cystic tumor (SCT), clinical and radiological features are diverse. Systematic classification of SCT subtypes is not well established. The purpose of this study was to classify the subtypes of SCT and to clarify its clinical and pathological characteristics. Clinical data from 52 patients with a pathologically confirmed SCT were prospectively collected using a standard data form. According to cyst size and multiplicity, on gross and radiological evaluation, the cysts were classified as microcystic when they were smaller than 2 cm, and macrocystic when larger than 2 cm. The microcystic tumors were subdivided into honeycomb and solid types, while the macrocystic tumors into unilocular and multilocular types based on the number of cysts. There were 22 cases with microcystic SCTs that were subclassified into the honeycomb (n = 21) and solid types (n = 1), while 30 cases were macrocystic type and were subclassified into multilocular (n = 16) and unilocular types (n = 14). There were no differences between four subtypes with regard to gender, tumor location, and size. The preoperative diagnostic accuracy of the unilocular macrocystic SCT was only 35.7%, while that of honeycomb microcystic SCT and multilocular macrocystic SCT were 81% and 87.5%, respectively (P = 0.005). Microcystic SCTs and multilocular macrocystic SCTs can be accurately diagnosed preoperatively; therefore conservative treatment and observation are possible in some cases. However, the unilocular macrocystic SCT is difficult to differentiate from the other pancreatic cystic tumors with malignant potential, therefore resection must be considered.

Unusual imaging appearances of pancreatic serous cystadenoma: correlation with surgery and pathologic analysis

We describe imaging and pathologic features of serous cystadenoma of the pancreas on multislice helical computed tomography CT (MS-CT) and surgical resection. Radiologic and pathologic features were analyzed in five patients. All patients underwent MS-CT and digital subtraction angiography (DSA), and four patients underwent magnetic resonance (MR) imaging. Preoperatively, three cases showed radiologic evidence of mainly solid appearance on MS-CT, and the suspected diagnoses were solid pancreatic tumors (patients 1-3). The other two cases showed radiologic evidence of macrocystic tumor of the pancreas, and the suspected diagnoses were mucinous cystic tumors (cases 4 and 5). All patients underwent surgery, and the diagnosis of serous cystadenoma was confirmed on pathologic examination. In three cases that showed a solid appearance on MS-CT, a microcystic appearance was identified on microscopic examination, and the tumors were found to be hypervascular lesions on multiphasic contrast-enhanced CT and DSA. In cases 1 and 2, the lesions showed high intensity with internal septation on T2-weighted MR images. In two cases, the tumors were classified as a macrocystic variant of serous cystadenoma, and no mural nodules, papillary projections, or calcifications were seen in the tumors. Imaging appearance of serous cystadenoma on MS-CT is various and sometimes indistinguishable from that of solid tumor or mucinous cystic tumors of the pancreas. Imaging findings of hypervascularity and a well-marginated high-intensity lesion with internal septation on T2-weighted MR imaging may be crucial to identify serous cystadenoma that contains no visible cystic compartments on MS-CT.

Cystic endocrine tumors of the pancreas: clinical, radiologic, and histopathologic features in 13 cases.

Cystic endocrine tumors of the pancreas are rare and raise difficult clinical problems. Our aims were to reevaluate the diagnostic and therapeutic strategy and to assess their histopathologic characteristics. Thirteen cystic endocrine tumors diagnosed in 10 patients were included. Clinical, radiologic, and pathologic data were reviewed. There were 6 male and 4 female patients (median age, 46 yrs). Six patients had evidence of multiple endocrine neoplasia type 1 (MEN1) disease. Four had a functional endocrine syndrome. Ten tumors were visible on imaging studies. The most suggestive radiologic features were the existence of a peripheral hypervascular rim (10 cases) and images of cyst into cyst (two cases). On gross and histologic examinations, two distinct types were present. Macrocystic tumors (six cases) were unilocular and limited by a thick wall containing nests of tumor cells. Microcystic tumors (seven cases) were characterized by the presence of multiple cystic spaces directly lined by tumor cells. Surgical resection was performed in all cases. Three patients had lymph node metastases at the time of diagnosis. One patient is dead with metastatic dissemination. The others are alive without recurrence or metastasis. The diagnosis of endocrine tumor must be considered for any pancreatic cyst discovered in a patient with a history of MEN1 syndrome or with clinical features suggestive of this syndrome. Cystic pancreatic endocrine tumors must be treated by surgical resection because of their possible malignant evolution.