Nonepithelial Tumors Research Articles

Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant Potential From the Radiologic Pathology Archives.

Almost all neoplasms of the pancreas are derived from pancreatic epithelial components, including the most common pancreatic mass, primary pancreatic ductal adenocarcinoma (PDAC). Nonepithelial neoplasms comprise only 1%-2% of all pancreatic neoplasms. Although some may arise directly from intrapancreatic elements, many originate from mesenchymal, hematopoietic, or neural elements in the retroperitoneal peripancreatic space and grow into the pancreas. Once these tumors reach a certain size, it can be challenging to identify their origin. Because these manifest at imaging as intrapancreatic masses, awareness of the existence and characteristic features of these nonepithelial neoplasms is crucial for the practicing radiologist in differentiating these tumors from primary epithelial pancreatic tumors, an important distinction given the vastly different management and prognosis. In part 1 of this article, the authors reviewed benign nonepithelial neoplasms of the pancreas. This article focuses on malignant nonepithelial neoplasms and those of uncertain malignant potential that can be seen in the pancreas. The most common malignant or potentially malignant nonepithelial pancreatic tumors are of mesenchymal origin and include soft-tissue sarcomas, solitary fibrous tumor, and inflammatory myofibroblastic tumor. These tumors commonly manifest as large heterogeneous masses, often containing areas of necrosis and hemorrhage. The clinical features associated with these tumors and the imaging characteristics including enhancement patterns and the presence of fat or calcification help distinguish these tumors from PDAC. Hematopoietic tumors, including lymphoma and extramedullary plasmacytoma, can manifest as isolated pancreatic involvement or secondarily involve the pancreas as widespread disease. Hyperenhancing paragangliomas or hypervascular metastatic disease can mimic primary pancreatic neuroendocrine tumors or vascular anomalies.

Primary Omental Gastrointestinal Stromal Tumor-What We Know and How Can We Deal With it, Based on Case Series and Review of Literature

Gastrointestinal stromal tumors (GIST) are considered uncommon mesenchymal spindle-cell or epithelioid neoplasm, featuring Cajal cell-like differentiation; arise in high frequency from the stomach or small bowel. GISTs represent the majority of primary non-epithelial tumors of digestive tract, most frequently expressing the KIT protein a transmembrane tyrosine kinase receptor for stem cell factor and KIT/PDGFRA mutation. A small percentage of GISTs originate from extragastrointestinal masses like omentum, mesentery, and retroperitoneum. These tumors are commonly named “extra-gastrointestinal GISTs” (EGIST) and tend to be present in less than 5%-10% of cases. Although the incidence of EGIST, their clinicopathologic parameters and clinical implications have yet to be defined because of the rarity of these tumors. Besides that, these neoplasms with overlapping immunohistological features, have no connection to the gastric or intestinal wall. We describe few cases of omentum compromise and all the associated features. Omentum EGIST can grow slowly and remain quite silent despite the size, which is commonly a large tumor. Immunohistological features tend to be slight different from GIST and the prognosis is generally worse. In most cases, preoperative diagnosis it is difficult to make and the surgery is needed. Moreover, the role of imatinib, the drug of choice in the treatment of this disease, it´s still unclear in EGIST. Alternative therapies are on the field showing good results.

THE CASE OF PRIMARY-MULTIPLE GONADAL TUMORS IN THE BACKGROUND OF THE SYNDROME OF COMPLETE INSENSITIVITY TO ANDROGENS

The article presents the case of rare combination of primary-multiple gonadal tumors: malignant disgerminoma and tumor that originating from Sertoli-Leydig cells with the syndrome of complete insensitivity to androgens. According to literature this kind of primary-multiple gonadal tumors are only 0,5%. Literature does not describe cases of such combination of malignant tumors and the syndrome of complete insensitivity to androgens. Proposed clinical observation demonstrates the need for inclusion in the risk groups for the development of gonadal tumors in girls with primary amenorrhea and dysgenesis of the gonads as well as the appropriateness and importance of medical genetic counseling in timely diagnosis and prevention of non-epithelial gonadal tumors.

Nonepithelial Tumors and Tumor-like Lesions of the Skin and Subcutis in Children.

This overview of mesenchymal tumors presenting in the skin and/or subcutis in children brings together the range of neoplasms and hamartomas which are seen in this age-group. It is not surprising from the perspective of the pediatric or general surgical pathologist that vascular anomalies, including true neoplasms and vascular malformations, are the common phenotypic category. Since there is considerable morphologic overlap among these lesions, clinicopathologic correlation may be more important than for many of the other mesenchymal tumors. The skin and subcutis are the most common sites of clinical presentation for the infantile myofibroma which is the most common of fibrous mesenchymal tumors in children. Several of the other mesenchymal tumors are more common adults-like dermatofibrosarcoma protuberans, but nonetheless have an important presence in children, even as a congenital neoplasm. A lipomatous tumor in a young child should be considered as a possible manifestation of an overgrowth syndrome.

Epigenetic silencing of lncRNA MORT in 16 TCGA cancer types

We have previously described a hominid-specific long non-coding RNA, MORT (also known as ZNF667-AS1, Gene ID: 100128252), which is expressed in all normal cell types, but epigenetically silenced during cancer-associated immortalization of human mammary epithelial cells. Initial analysis of The Cancer Genome Atlas (TCGA) showed that 15 of 17 cancer types, which represent the 10 most common cancers in women and men, display DNA methylation associated MORT silencing in a large fraction of their tumors. In this study we analyzed MORT expression and DNA methylation state in the remaining 16 TCGA cancer types not previously reported. Seven of the 16 cancer types showed DNA methylation linked MORT silencing in a large fraction of their tumors. These are carcinomas (cervical cancer, and cancers of esophagus, stomach, and bile duct), and the non-epithelial tumors mesothelioma, sarcoma, and uterine carcinosarcoma. Together with the findings from our previous report, MORT expression is silenced by aberrant DNA methylation in 22 of 33 of TCGA cancer types. These 22 cancers include most carcinoma types, blood derived cancers and sarcomas. In conclusion, results suggest that the MORT gene is one of the most common epigenetic aberrations seen in human cancer. Coupled with the timing of MORT gene silencing during in vitro epithelial cell immortalization and its occurrence early in the temporal arc of human carcinogenesis, this provides strong circumstantial evidence for a tumor suppressor role for MORT.

Epigenetic silencing of lncRNA MORT in 16 TCGA cancer types.

We have previously described a hominid-specific long non-coding RNA, MORT (also known as ZNF667-AS1, Gene ID: 100128252), which is expressed in all normal cell types, but epigenetically silenced during cancer-associated immortalization of human mammary epithelial cells. Initial analysis of The Cancer Genome Atlas (TCGA) showed that 15 of 17 cancer types, which represent the 10 most common cancers in women and men, display DNA methylation associated MORT silencing in a large fraction of their tumors. In this study we analyzed MORT expression and DNA methylation state in the remaining 16 TCGA cancer types not previously reported. Seven of the 16 cancer types showed DNA methylation linked MORT silencing in a large fraction of their tumors. These are carcinomas (cervical cancer, and cancers of esophagus, stomach, and bile duct), and the non-epithelial tumors mesothelioma, sarcoma, and uterine carcinosarcoma. Together with the findings from our previous report, MORT expression is silenced by aberrant DNA methylation in 22 of 33 of TCGA cancer types. These 22 cancers include most carcinoma types, blood derived cancers and sarcomas. In conclusion, results suggest that the MORT gene is one of the most common epigenetic aberrations seen in human cancer. Coupled with the timing of MORT gene silencing during in vitro epithelial cell immortalization and its occurrence early in the temporal arc of human carcinogenesis, this provides strong circumstantial evidence for a tumor suppressor role for MORT.

Trends in Gastric Cancer Incidence According to the Clinicopathological Characteristics in Korea, 1999-2014.

PurposeThe aim of this study was to evaluate recent trends in gastric cancer incidence according to the age, sex, and tumor location in the Korean population. Materials and MethodsUsing data from the Korea Central Cancer Registry between 1999 and 2014, gastric cancer incidence, annual percent changes, and male-to-female incidence rate ratios (IRRs) according to tumorlocationwere determined. The distribution of disease extent according to the tumor location and its changes between 2006 and 2014 were also analyzed.ResultsIncidence of gastric cancer was stable until 2011 and decreased between 2011 and 2014. The age-standardized incidence rate of gastric cancer was 43.6 (per 100,000) in 1999 and 35.8 in 2014. The proportion of cardia/fundus cancer remained stable (5.9% to 7.1%), and that of body cancer increased (35.3% to 43.2%). The male-to-female IRR decreased in most age groups, except for those in their 60s. In the distribution of disease extent, the proportion of localized disease increased, and regional and distant disease decreased in all tumor locations (53.9% to 66.0%, 31.4% to 22.5%, and 14.8% to 11.5%, respectively; p < 0.001). For histological type, the proportion of carcinoid tumor and non-epithelial tumor increased (0.3% to 1.0%, and 0.8% to 1.4%, respectively). ConclusionIn the 15 years from 1999 through 2014, age-standardized incidence of gastric cancer started to decrease from 2012, and the proportion of cardia/fundus cancer remained unchanged. The trend of increasing localized cancer was observed in all tumor locations.

Esrp1 is a marker of mouse fetal germ cells and differentially expressed during spermatogenesis.

ESRP1 regulates alternative splicing, producing multiple transcripts from its target genes in epithelial tissues. It is upregulated during mesenchymal to epithelial transition associated with reprogramming of fibroblasts to iPS cells and has been linked to pluripotency. Mouse fetal germ cells are the founders of the adult gonadal lineages and we found that Esrp1 mRNA was expressed in both male and female germ cells but not in gonadal somatic cells at various stages of gonadal development (E12.5-E15.5). In the postnatal testis, Esrp1 mRNA was highly expressed in isolated cell preparations enriched for spermatogonia but expressed at lower levels in those enriched for pachytene spermatocytes and round spermatids. Co-labelling experiments with PLZF and c-KIT showed that ESRP1 was localized to nuclei of both Type A and B spermatogonia in a speckled pattern, but was not detected in SOX9+ somatic Sertoli cells. No co-localization with the nuclear speckle marker, SC35, which has been associated with post-transcriptional splicing, was observed, suggesting that ESRP1 may be associated with co-transcriptional splicing or have other functions. RNA interference mediated knockdown of Esrp1 expression in the seminoma-derived Tcam-2 cell line demonstrated that ESRP1 regulates alternative splicing of mRNAs in a non-epithelial cell germ cell tumour cell line.

Fertility Sparing Management in Non-Epithelial Ovarian Cancer. Which Patients, What Procedure and What Outcome?

Non epithelial ovarian tumors represent a small group of rare gynecological cancers but they have a high incidence in young childbearing women. The recent years fertility preservation surgeries have become a common practice in specific ovarian malignancies when the patients wish to maintain their fertility. Specific types of non-epithelial ovarian tumors can be managed with fertility sparing operations with a similar outcome to more radical intervention but due to the rarity of these tumors the extent of the operation remains in some cases controversial. Moreover, the reproductive outcome of the women that had these conservative operations seems to be very promising. In our review we try to summarize the data regarding the fertility sparing management of all types of non-epithelial ovarian cancers and what procedure should be performed in each case. Finally we have accumulated the data concerning the reproductive outcome of patients that had undergone this type of surgery.

Clinical and Histopathological Characteristics of Gastric Adenocarcinoma in Yemeni Patients: A 2 Years Prospective Study

Objectives: To study the clinical and the histopathological characteristics of gastric cancer concentrating more on the gastric adenocarcinoma. Design: Prospective study of one hundred thirty Yemeni patients confirmed with gastric malignancies during the period from July 2016 to July 2017. Setting: Specialized center of gastrointestinal and liver diseases in Ibb city, Yemen. Subjects: All patients with histopathologically proved gastric cancer. Results: We collect 130 patients with gastric cancer during the study period, they were 93 males and 37 females with age ranged between 25 years and 100 years and the mean age was 64.98 ± 15.15 years. The most frequent clinical presentations of our patients were epigastric pain (81.5%), weight loss (74.6%), dysphagia (46.9%), vomiting (49.2%), palpable epigastric mass (19.2%) and hematemesis/melena (20%). Proximally located gastric cancer represented 46.1%; 48.9% of males patients and 38.9% of females patients. Adenocarcinoma constituted 92.4%, of which the intestinal type was 82.5% and the diffuse type was 17.5%. Squamous cancer accounted in 1.5%, and the non-epithelial tumor was in 6.1%, of which 4.6% were lymphoma and 1.5% were GIST. Conclusion: Adenocarcinomas are the major histological type of gastric cancer and represent 92.4% of all gastric malignancies in which intestinal type represented 82.5% and diffuse type represented 17.5%, and proximally located gastric cancer within stomach was the commonest in both sexes.

Clinicopathological Pattern of Gastric Cancer of Yemeni Patients in Ibb Governorate

Objectives: To study the clinicopathological pattern of gastric cancer in-cluding the location of the tumor in the stomach and to compare our results with other studies. Design: Prospective study of patients confirmed with gastric malignancy during the period from July 2016 to July 2017. Setting: Specialized center of gastrointestinal and liver diseases in Ibb city, Yemen. Subjects: All patients with histopathological confirmation of gastric malig-nancy. Results: During the study period, we had 69 patients with gastric cancer; among them 48 were males and 21 were females, with male: Female ratio is 2.2:1. The age ranged between 35 years and 90 years and the mean age was 65.87 ± 12.79 years. Most of the patients presented with epigastric pain (87%), weight loss (72.5%), pallor (65.2%), dysphagia (47.8%), vomiting (42%), palpable epigastric mass (20.3%) and hematemesis/melena (18.8%). The cardiofundal location was encountered in 33 cases (47.8% ), 22 (45.8%) males and 11 (52.4%) females, the corpus location in 24 cases (26%), 20 (41.7%) males and 4 (19%) females, the antral location in 8 cases (11.6%), 4 (8.3%) males and 4 (19%) females and the whole stomach in 4 cases (5.8%), 2 (4.2%) males and 2 (9.5%) females. Adenocarcinoma constituted about 94.2%, of which the intestinal type was 87.5% and the diffuse type was 12.5%. The non-epithelial tumor was encounter 4.3%, of which 2.9% were lymphoma and 1.4% were GIST. Conclusion: In our studied patients, proximally located gastric cancer was the most common in both sexes.

Primary pulmonary sarcoma - long-term treatment outcomes and prognostic factors.

IntroductionPrimary pulmonary sarcoma (PPS) is a rare type of non-epithelial malignant tumour of the lungs. Lung sarcomas are usually of metastatic character, originating from the primary tumour located in another organ. Pathomorphological diagnosis is difficult and therefore definitive diagnosis is usually determined after surgical treatment. Five-year survival of radical operated patients is 42–69%.AimTo evaluate prognostic factors in PPS.Material and methodsTwenty-two patients who underwent surgical treatment between 1994 and 2009 were analysed.ResultsTen types of sarcomas were identified. All 22 analysed patients underwent operations and in 14 (63.6%) cases it was R0 resection (pneumonectomies, lobectomies, non-anatomical resections). In 8 out of 22 patients a locally advanced form was identified. Median overall survival (OS) in the group of all patients was 22 months, 5- and 10-year OS was 27% and 18% respectively, whereas in patients who underwent non-anatomical lung resections it was 80% and 60% respectively with median OS of 213 months. Overall survival was dependent on gender, completeness of treatment and size of tumour. Haemoptysis was a poor prognostic factor. Primitive neuroectodermal tumour was a type of sarcoma with a negative effect on progression-free survival. No impact of age, type of sarcoma, degree of histological malignancy, stage or adjuvant treatment on OS was noted.ConclusionsPrimary radical surgical resection prolongs patient survival. Patients with tumours larger than 5 cm and men have worse prognoses. Future studies conducted on larger groups of patients diagnosed with PPS are necessary.

Extracapsular excision of hepatic hemangioma: A single centre experience.

Backgrounds/AimsHepatic hemangioma is a common non-epithelial neoplasm of the liver. Presence of symptoms and uncertainty in diagnosis are the most common indications for surgery.MethodsEighteen patients with hepatic hemangioma, operated on from January 2011 to December 2016 at the Hepato-pancreato-biliary surgical unit of Tata Memorial Hospital, were retrospectively analyzed.ResultsMain indications for operation were presence of symptoms, the most common being pain and diagnostic uncertainty. The median size of hemangioma was 9.9 cm (range 3.2 to 24 cm). All patients underwent extra-capsular excision of hemangioma. The median operating time was 180 minutes (range 75 to 460 minutes) and median blood loss was 950 ml (range 100 to 3,500 ml). Median post-operative stay was 5.5 days (range 3 to 10 days). One (5.6%) patient required re-exploration for post-operative hemorrhage, Clavien Dindo (CD) grade IIIb, and one (5.6%) had postoperative purulent intra-abdominal collection requiring percutaneous cutaneous drainage CD grade IIIa. There was no postoperative mortality. Postoperative day 3 liver function tests were within normal limits. Size of the tumor did not correlate significantly with postoperative complications (p=0.135).ConclusionsSurgical treatment of hemangioma should be guided by presence of symptoms or by the presence of diagnostic uncertainty, not by size alone. The size had no correlation with perioperative complications. The technique of extra-capsular excision is safe and technically feasible in most of the hemangiomas. This technique preserves maximum liver parenchyma, resulting in early postoperative recovery with minimal morbidity.

A practical diagnostic approach to hepatic masses.

The differential diagnosis of hepatic mass lesions is broad and arriving at the right diagnosis can be challenging, especially on needle biopsies. The differential diagnosis of liver tumors in children is different from adults and is beyond the scope of this review. In adults, the approach varies depending on the age, gender, and presence of background liver disease. The lesions can be divided broadly into primary and metastatic (secondary), and the primary lesions can be further divided into those of hepatocellular origin and nonhepatocellular origin. The first category consists of benign and malignant lesions arising from hepatocytes, while the second category includes biliary, mesenchymal, hematopoietic, and vascular tumors. Discussion of nonepithelial neoplasms is beyond the scope of this review. The hepatocytic lesions comprise dysplastic nodules, focal nodular hyperplasia, hepatic adenoma, and hepatocellular carcinoma, and the differential diagnosis can be challenging requiring clinicopathological correlation and application of immunohistochemical (IHC) markers. Liver is a common site for metastasis, sometimes presenting with an unknown primary site, and proper workup is the key to arriving at the correct diagnosis. The correct diagnosis in this setting requires a systematic approach with attention to histologic features, imaging findings, clinical presentation, and judicious use of IHC markers. The list of antibodies that can be used for this purpose keeps on growing continually. It is important for pathologists to be up to date with the sensitivity and specificity of these markers and their diagnostic role and clinical implications. The purpose of this review is to outline the differential diagnosis of hepatic masses in adults and discuss an algorithmic approach to make a right diagnosis.

Treatment of posterior gastric wall gastrointestinal stromal tumor with gastric sleeve: a case report

Gastrointestinal stromal tumors (GISTs) are the most common non-epithelial tumors of the gastrointestinal tract 1 . The most usual location is the stomach, followed by the small intestine, where it may cause digestive bleeding and anemia 6 . Surgical resection of the tumor is the gold standard treatment, and definitive diagnosis is based on immunohistochemical analysis of the surgical specimen 8 . We report the case of a 53-year-old man with gastric GIST presenting with endophytic and exophytic growth, located at the posterior wall of the stomach, in the antrum-body transitional zone, treated with gastric sleeve. Keywords: Gastrointestinal stromal tumors; gastrointestinal neoplasms; gastric sleeve; diagnosis; prognosis; treatment

Colonic intussusception caused by an ulcerating lipoma in an adult: A rare case report and literature review

Intestinal lipomas are rare nonepithelial tumors that are typically small in size and detected incidentally. Colonic intussusception is an uncommon complication of colonic lipoma. A 56 y old woman visited our emergency room with sudden onset of intermittent abdominal cramps. A CT scan showed a 5.0 cm, nodular, homogenous, hypodense structure in the hepatic flexure of the colon as the lead point of an intussusception. The hypodense structure was suggestive of a colonic lipoma, and the tumor was suspected to be the cause of the intestinal intussusception. Laparoscopic exploration was performed, and the pathology demonstrated a mature submucosal colonic lipoma. In addition to the case report, we discuss the role of imaging modalities in diagnosing neoplasms and for assessing the clinical behavior of lipomas when symptomatic.

Possibility of women treated with fertility-sparing surgery for non-epithelial ovarian tumors to safely and successfully become pregnant-a Chinese retrospective cohort study among 148 cases.

This study was performed to evaluate the oncological and reproductive outcomes of childbearing-age women treated with fertility-sparing surgery (FSS) for non-epithelial ovarian tumors in China. One hundred and forty eight non-epithelial ovarian tumor women treated with FSS between January 1, 2000 and August 31, 2015 from two medical centers in China were identified. Progression-free survival (PFS) was 88.5%, whereas overall survival (OS) was 93.9%. Univariate analysis suggested that delivery after treatment is related to PFS (P = 0.023), whereas histology significantly influenced OS. Cox regression analysis suggested that only histology was associated with PFS and OS (P < 0.05). Among the 129 women who completed adjuvant chemotherapy (ACT), none developed amenorrhea. Among the 44 women who desired pregnancy, 35 (79.5%) successfully had 51 gestations including 35 live births without birth defects. Non-epithelial ovarian tumors can achieve fulfilling prognosis after FSS and chemotherapy. Histology might be the only independent prognostic factor for PFS and OS. FSS followed by ACT appeared to have little or no effect on fertility. Meanwhile, postoperative pregnancy did not increase the PFS or OS. Use of gonadotropin-releasing hormone agonist was not beneficial for fertility.

Development of effective tumor immunotherapy using a novel dendritic cell-targeting Toll-like receptor ligand.

Although dendritic cell (DC)-based immunotherapy shows little toxicity, improvements should be necessary to obtain satisfactory clinical outcome. Using interferon-gamma injection along with DCs, we previously obtained significant clinical responses against small or early stage malignant tumors in dogs. However, improvement was necessary to be effective to largely developed or metastatic tumors. To obtain effective methods applicable to those tumors, we herein used a DC-targeting Toll-like receptor ligand, h11c, and examined the therapeutic effects in murine subcutaneous and visceral tumor models and also in the clinical treatment of canine cancers. In murine experiments, most and significant inhibition of tumor growth and extended survival was observed in the group treated with the combination of h11c-activated DCs in combination with interferon-gamma and a cyclooxygenase2 inhibitor. Both monocytic and granulocytic myeloid-derived suppressor cells were significantly reduced by the combined treatment. Following the successful results in mice, the combined treatment was examined against canine cancers, which spontaneously generated like as those in human. The combined treatment elicited significant clinical responses against a nonepithelial malignant tumor and a malignant fibrous histiocytoma. The treatment was also successful against a bone-metastasis of squamous cell carcinoma. In the successful cases, the marked increase of tumor-responding T cells and decrease of myeloid-derived suppressor cells and regulatory T cells was observed in their peripheral blood. Although the combined treatment permitted the growth of lung cancer of renal carcinoma-metastasis, the marked elevated and long-term maintaining of the tumor-responding T cells was observed in the patient dog. Overall, the combined treatment gave rise to emphatic amelioration in DC-based cancer therapy.

Treatment Approach and Prognosis of Pediatric and Adolescent Nonepithelial Malignant Ovarian Tumors: A Retrospective Prognosis Analysis.

Nonepithelial malignant ovarian tumors are rare in the pediatric and adolescent population. The aim of this study was to observe the spectrum of pathology, presentation, outcome, and risk factors for survival of pediatric nonepithelial malignant ovarian tumors in a Chinese pediatric population. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: This was a retrospective study of 171 girls (median age at presentation of 14years) diagnosed with primary malignant ovarian tumors between 1990 and 2014 at the Yat-Sen Memorial Hospital and Cancer Center of Sun Yat-sen University. Symptoms, pathological data, treatments, and outcomes were obtained retrospectively from the medical records. Most (85.96%, 147/171) tumors occurred in patients aged 10-18years and most cases were International Federation of Gynecology and Obstetrics stage I (68.42%, 117/171). The predominant pathological type was germ cell tumors (87.13%, 149/171). All patients underwent surgery, and 87 (50.88%, 87/171) underwent conservative incomplete staging surgery (unilateral salpingo-oophorectomy or tumor excision). The 5-year progression-free survival (PFS) was 59.2%. The 5-year overall survival (OS) was 88.7%. Surgical hospital (hazard ratio, 0.388; 95% confidence interval, 0.213-0.706; P=.002) was independently associated with PFS. Recurrence state (hazard ratio, 163.26; 95% confidence interval, 1.321-20,181.875; P=.038) was independently associated with OS. Ovarian cancers in children and adolescents have features of good prognosis. Girls who received their first surgery in a tertiary hospital had better PFS. Patients who did not suffer recurrence had better OS.

Metastatic Gastrointestinal Stromal Tumor Disguising as Pancreatic Pseudocyst

Gastrointestinal Stromal Tumors (GISTs) are the most common non-epithelial benign tumor of the gastrointestinal tract. We present a patient with abdominal pain previously diagnosed with pancreatic pseudocyst in which etiology was ultimately determined to be due to metastatic GISTs. A 59-year-old African American female with past medical history of hypertension, and diabetes mellitus presented to the Emergency room with diffuse abdominal pain for a month. Patient also reported early satiety, nausea and 50 lbs weight loss over the past 2-3 months. She initially presented about a month ago to an out-of-state hospital with abdominal pain. Evaluation revealed a large pancreatic pseudocyst. She underwent, an endoscopic ultrasound guided cystogastrostomy with placement of stent. She continued to have abdominal pain, she was currently in town visiting family, but because of worsening abdominal pain she presented to the emergency room. Imaging of the abdomen had revealed a very large abdominal mass between the gastric body and pancreatic body and tail. There was also a lymph node lesion. She underwent an endoscopic ultrasound, which revealed an ulcerated mass 3.5 cm in size in the proximal stomach along the lesser curvature. On the contralateral gastric wall, in the mid gastric body, there was the previously placed stent, and an attempt to the scope through the stent was unsuccessful with marked friable tissue ingrowth. There was no cystic cavity. The endoscopic ultrasound noted a very large hypoechocic mass at least 8 X 9 cm in size, appearing to arise from either pancreatic body or gastric wall with invasion into the gastric wall. There was also 2.3 cm hypoechoic mass in the left lobe of the liver. All the above lesions have been biopsied and results showed a high-grade malignant spindle cell neoplasm consistent with GISTs. Patient was referred to Oncology and Surgery for further care and management. GISTs are the most common non-epithelial benign tumor of the gastrointestinal tract. They are most commonly found in the stomach and small intestine. Our patient was previously diagnosed with pancreatic pseudocyst for which she underwent endoscopic drainage with negative workup for malignancy. Tumor markers including CEA, CA19-9 and AFP were normal as well. This Case illustrates that GIST should be considered in the differential diagnosis when a patient presenting with pancreatic pseudocyst.