Large Bowel Tumors Research Articles

Carcinoid Heart Disease.

Carcinoid heart disease (CHD) is a rare cardiac complication that occurs most commonly in patients with advanced neuroendocrine tumors and is a known sequela of carcinoid syndrome. Neuroendocrine tumors most widely associated with CHD include tumors in the small bowel, followed by lung, large bowel, pancreatic, appendiceal, and ovarian neoplasms. Carcinoid syndrome is a paraneoplastic syndrome caused by the release of serotonin and other substances from neuroendocrine tumors. It results in a spectrum of symptoms, including diarrhea, flushing, bronchospasm, and symptoms of congestive heart failure. Without treatment and for patients with advanced heart failure, the prognosis of CHD can be less than a year. Management of CHD is often challenging as patients typically present late, and the disease can progress rapidly. Therefore, optimal management of these patients requires close collaboration among various specialties to quantify disease burden, delay the progression of valvular disease, and determine the most effective surgical and medical management strategies depending on the cardiac manifestations to improve quality of life and reduce mortality. This involves a collaborative team, including cardiology and oncology, and often involves many other disciplines, including hepatobiliary and cardiovascular surgeons, endocrinologists, anesthesiologists, and gastroenterologists.

Immunoreactivity of HOXB13 in Neuroendocrine Neoplasms Is a Sensitive and Specific Marker of Rectal Well-Differentiated Neuroendocrine Tumors.

HoxB13 is a transcription factor involved in defining of posterior endodermal derivatives, including prostate and rectum. While it is used as a marker of prostatic adenocarcinoma, it has not been studied systematically in neuroendocrine neoplasms. Thus, we performed HoxB13 immunohistochemistry in tissue microarrays and the whole sections of 232 neuroendocrine neoplasms. These included 34 paragangliomas (PGs), 20 cauda equina neuroendocrine tumors (CENETs), 123 well-differentiated neuroendocrine tumors (WDNETs), and 55 neuroendocrine carcinomas (NECs). WDNETs were additionally analyzed with SATB2, and colorectal WDNETs with CDX2 and serotonin immunohistochemistry. In total, HoxB13 immunoreactivity was observed in 95% (19/20) CENETs, 10.6% (13/123) WDNETs, and 12.9% (7/54) NECs. No PGs were positive. Large intestine WDNETs expressed HoxB13 in 68.4% (13/19); five negative tumors originated in cecum and one in rectum. In rectum, 92.9% (13/14) WDNETs expressed HoxB13. HoxB13 was 92.9% sensitive and 100% specific, showing 100% positive predictive value for the rectal origin of WDNET. In NECs, HoxB13 was positive in 15.4% (2/13) GIT tumors and 80% (4/5) prostatic NECs, but in none of urinary bladder NECs (0/8). SATB2 was positive in 17.1% (21/123) WDNETs, including 78.9% (15/19) of colorectal WDNETs, 71.4% (5/7) appendiceal WDNETs, and 2.9% (1/34) small intestine WDNETs. All 4 SATB2-negative large bowel tumors originated in the cecum. When both markers combined, HoxB13+/SATB2+ immunoprofile was seen exclusively in rectal WDNETs (positive predictive value 100%), while HoxB13-/SATB2+ immunoprofile was highly suggestive of the appendiceal origin (positive predictive value 71.4%). Therefore, HoxB13 can be useful as an immunohistochemical marker of rectal WDNETs and prostatic NECs.

Giant lipoma of the colon presenting as a case of adult intussusception: A case series

Introduction and importanceLipoma of the intestine is one of the uncommon benign tumors of the alimentary tract first reported by Bauer in 1957. The peak incidence is usually seen between 50 and 60 years of age and more commonly in females. They are usually either asymptomatic or mildly symptomatic. The occurrence of symptoms is mostly related to the diameter of the lesion. Presentation of caseWe present a single center consecutive case of three patients who had giant colonic lipomas presented with colonic intussusception. Two of the cases presented for the first time with an emergency condition: acute intestinal obstruction. The mode of presentation, diagnosis, and management outcome of colonic lipoma was assessed. DiscussionSymptomatic lipoma may present with non-specific abdominal pain, change in bowel habits, intussusception, and hemorrhage. The clinical diagnosis is usually challenging, given the non-specificity of symptoms of the disease. Computed tomography is considered to be a diagnostic modality of choice in recognizing the presence of lipoma. However, a definitive diagnosis of lipoma is usually obtained after a histopathologic examination of the resected specimen. The management of colonic lipoma depends on the size of the lesion and the presence or absence of symptoms. ConclusionColonic lipoma is a rare benign tumor that occurs in the elderly, frequently misdiagnosed as a malignant tumor. Despite the rarity of the disease, lipoma should be considered in the differential diagnosis of large bowel tumors and adult intussusception.

Large Bowel's tumor of unclear histogenetic origin from the group of neuroendocrine tumors with life-threatening hemorrhage and hemorrhagic shock

Introduction. Neuroendocrine neoplasms (NENs) of the gastrointestinal tract (GIT) are slow-growing and rare tumors with different clinical, histological, and biological characteristics with an increased incidence in recent years. Most of them are indolent and colonic NENs are rare among all GIT-NENs. Compared to colorectal adenocarcinoma neuroendocrine tumors of the colon and sigmoid colon are uncommon. Case outline. We present a 25-year-old female patient, who was admitted to our department in hemorrhagic shock due to life-threatening bleeding from a tumor on the sigmoid colon, and after unsuccessful endoscopic hemostasis during colonoscopy. The complaints started the day before admission to the ward with hematochezia. The patient had no complaints before that. Emergency operation and colon resection with terminal colostomy were performed. Pathohistological and immunohistochemical analysis of the tumor showed unclear histogenetic origin from the group of neuroendocrine tumors. Conclusion. Regardless of the asymptomatic period of the disease, these tumors can cause severe bleeding as the first symptom, which can be life-threatening.

Virtual Chromoendoscopy in Capsule Endoscopy: A Narrative Review.

The usefulness of virtual chromoendoscopy (VC) in capsule endoscopy (CE) isa controversial issue, with conflicting studies regarding its efficacy. FICE and a blue filter were embedded in the PillCamTM software, with the aim to assist readers in identifying the source of obscure gastrointestinal (GI) bleeding (OGIB), coeliac disease mucosal changes and other small and large bowel lesions, including polyps and tumors. This review aims to summarize the existing evidence on the value of VC in the visualization and identification of different types of pathology. Overall, VC in CE with FICE 1 and 2 can be a useful adjunctive tool and may increase the visibility of pigmented lesions, such as angiectasias and ulcers. However, it does not appear to improve the detection of polyps or tumors. On the other hand, the role of FICE 3 and the blue filter appears to be limited. FICE may also be helpful in differentiating hyperplastic and adenomatous colonic polyps during colon capsule endoscopy, although more evidence is needed.

Blood Serum Zonulin in Colorectal Cancer, Autoimmune Bowel Diseases, and Irritable Bowel Syndrome.

Zonulin content in blood serum of patients with colorectal cancer (CRC; n=152; 30-84 years) and patients with large bowel adenomas (n=32; 39-82 years) was measured by standardized kit IDK Zonulin ELISA (Immundiagnostik AG). The healthy control group (n=50) comprised volunteers (27 women, 23 men; 25-68 years); pathological control group (n=84) - patients (55 women, 29 men;18-84 years) with irritable bowel syndrome (n=29), Crohn's disease (n=5), and ulcero-necrotic colitis (n=50). In comparison to healthy control group, the level of zonulin was significantly increased in CRC patients (p<0.0000001) and in patients with benign large bowel tumors (p<0.004), as well as in patients with inflammatory intestine diseases and with irritable bowel syndrome (p<0.0002). Zonulin level in blood serum of CRC patients was slightly, but significantly higher (p<0.05) than in the group of pathological control. ROC curve construction revealed that at optimal zonulin cut-off level (52.2 ng/ml), the diagnostic sensitivity of CRC detection was 66.7% and specificity relative to healthy control was 81.8%. The specificity relative to the combined control group (healthy control+non-tumor bowel diseases) was only 68.9%. Thus, no acceptable cut-off levels for differentiation between malignant and benign tumors, as well as between tumor and non-tumor large bowel pathologies were found. Analysis of the associations between serum zonulin level and the main clinical and pathological characteristics of CRC demonstrated that the level of this marker increased with disease progression (p<0.01; Kruskal-Wallis test), but was not associated with individual criteria of the TNM system, tumor localization, histological structure, and malignancy grade.

Possibilities of the indocyanine green dye application for evaluation of peculiarities of lymph outflow and metastasizing of colorectal cancer

Objective. Studying of velocity of prevalence of luminescent dye indocyanine green along lymphatic vessels in the large bowel surgery.&#x0D; Materials and methods. Estimation of procedure for coloring of tumoral substrate and lymphatic vessels while performance of oncological operations was conducted. We performed laparoscopic resection of sigmoid colon in 7 patients, suffering tumors, and anterior rectal resection in 5 patients, having tumors of upper ampullary part of rectum, using indocyanine green dye.&#x0D; Results. In Group I, consisting of 7 patients, surgical technique of no touch manipulations on the tumor before transection of feeding vessels (NTIT–operations) was applied, and in Group II, consisting of 5 patients, the tumor mobilization was done primarily. Application of indocyanine green dye helped a lot to establish localization and prevalence of the process in rectum.&#x0D; Conclusion. Application of fluorescent dye indocyanine green on tumoral substrate before doing ligation of feeding vessels raises possibility of the complication occurrence, consisting of the cancer cells dissemination in abdominal cavity. While performing laparoscopic procedures in resection of the large bowel tumors it is expedient to ligate the feeding vessels primarily, with secondary manipulation– to dissect and mobilize the tumoral substrate.

Primary Pediatric Non-Hodgkin Lymphomas of the Gastrointestinal Tract: A Population-based Analysis.

The aim of this study was to present the clinical characteristics, natural history and survival outcomes of primary gastrointestinal non-Hodgkin lymphomas (PGINHL) in the pediatric population. Surveillance, Epidemiology, and End Results (SEER) database was queried for patients aged 0 to 19 years with PGINHL between 1973 and 2014. A total of 452 cases were identified [mean age 11.0 (±5.1)] years, whites 84.1%, males (76.5%). The majority of tumors were noted in the small bowel (SB) (47.6%), followed by large bowel (LB) (28.5%) and the stomach (10.0%). Overall, the most common histological subtype was Burkitt lymphoma (51.8%), followed by diffuse large B-cell lymphoma (DLBCL) (26.1%). Mean overall survival (OS) of the entire cohort was 33,33 years with a 5-yr, 10-yr and 30-yr survival rate of 86%, 86% and 79%, respectively. Large bowel tumors had the best long-term survival rates whereas; gastric tumors had the worst with 30-yr survival rate 84% and 74%, respectively. Overall, 328 (72.6%) patients received surgery. No significant survival difference was noted between patients who underwent surgery and those who did not. This study presents the largest dataset of pediatric PGINHL and describes the clinical features and outcomes of these patients in addition to summarizing the literature.

Morphology and Molecular Features of Rare Colorectal Carcinoma Histotypes.

Several histopathological variants of colorectal carcinoma can be distinguished, some associated with specific molecular profiles. However, in routine practice, ninety/ninety-five percent of all large bowel tumors are diagnosed as conventional adenocarcinoma, even though they are a heterogeneous group including rare histotypes, which are often under-recognized. Indeed, colorectal cancer exhibits differences in incidence, location of tumor, pathogenesis, molecular pathways and outcome depending on histotype. The aim is therefore to review the morphological and molecular features of these rare variants of intestinal carcinomas which may hold the key to differences in prognosis and treatment.

Primary low-grade fibromyxoid sarcoma of the large intestine with lung metastases: A case report and review of the literature

A 70-year old man had multiple lung nodules detected on a computed tomography (CT) performed as part of the work-up for an isolated episode of hematuria of several hours' duration. A CT-guided lung biopsy disclosed a morphologically undifferentiated neoplasm, that could not be further subclassified despite an extensive immunohistochemical panel.Four years previously the patient had undergone a left hemicolectomy in another institution to resect a 4.7 cm polypoid ulcerated area in the splenic flexure. The slides from that resection were retrieved for review at our institution. There was a variably cellular, diffuse spindle cell neoplastic proliferation involving the submucosa, muscularis propria and subserosa of the large bowel, composed of elongated spindle cells, with small, inconspicuous nucleoli, arranged in intersecting fascicles. Occasional areas of the tumor showed myxoid stroma. Mitotic activity was low.Immunohistochemistry showed strong and diffuse cytoplasmic staining for MUC4 in both the large bowel and lung tumors. Molecular profiling identified a FUS-CREB3L1 fusion gene.The constellation of morphological, immunohistochemical and molecular features are of low-grade fibromyxoid sarcoma (LGFMS) of the large bowel, with metastatic fibromyxoid sarcoma to the lung.LGFMS is a malignant, often late-metastasizing tumor, with a misleadingly bland histological appearance, that typically arises in the deep soft tissues of the proximal extremities or trunk of young adults. Accurate classification, requiring morphology and ancillary tests, is required for appropriate clinical follow-up and treatment of patients.

A Myth in Colonoscopy: Cushion Sign or Pillow Sign

Colonic lipomas are benign non-epithelial adipocyte hyperplasia of the gastro-intestinal tract. They are the second common begin large bowel tumor after adenomatous polyps. Pillow sign is defined as indentation of colonic tumors with the use of the biopsy forceps during colonoscopy. Here, we report a 69 -year- old healthy female with pillow sign in screening colonoscopy. A 69 year old female with no significant past medical history was referred from her primary care physician for age appropriate screening colonoscopy. Physical examination and routine labs were within reference ranges. After optimal bowel preparation, she underwent screening colonoscopy. On colonoscopy, a 3 cm lipomatous lesion was seen in the ascending colon with a positive “pillow sign” or “cushion sign”. Multiple diverticuli are found in the descending colon and sigmoid colon. Colonic lipomas are accidentally found during diagnostic or screening colonoscopy. They are usually asymptomatic but can sometimes present with complications such as vague abdomen pain, bleeding per rectum, intestinal obstruction, or intussusception particularly in lipoma larger than 2 cm. Management depends on the size and presenting symptoms .However, there is no proper guideline to follow up for those less than 2 cm asymptomatic colonic lipomas. The size of the stalk is more important than the size of lipoma in considering endoscopic removal.2931 Figure 1. A 3 cm lipomatous lesion seen in the ascending colon with a positive “Pillow sign”

Current Clinical Indications for Small Bowel Capsule Endoscopy.

Small bowel capsule endoscopy is currently the first line diagnostic examination for many diseases affecting the small bowel. This article aims to review and critically address the current indications of small bowel capsule endoscopy in clinical practice. Bibliographic review of relevant and recent papers indexed in PubMed. Small bowel capsule endoscopy enables a non-invasive full-assessment of the small bowel mucosa, with high diagnostic yield even for subtle lesions. In patients with obscure gastrointestinal bleeding, diagnostic yield is higher when performed early after the onset of bleeding. Endoscopic treatment of angioectasias using balloon-assisted enteroscopy may contribute to reduce rebleeding, while the risk of rebleeding in patients with 'negative' small bowel capsule endoscopy is debatable. Cross-sectional imaging may be more accurate than small bowel capsule endoscopy for the diagnosis of large small bowel tumors. The Smooth Protruding Index on Capsule Endoscopy (SPICE score) may help to differentiate submucosal tumors from innocent bulges. Small bowel capsule endoscopy is also a key diagnostic instrument in patients with suspected Crohn's disease and non-diagnostic ileocolonoscopy; it may also influence prognosis and therapeutic management, by determining disease extent and activity in patients with known Crohn's disease. The role of small bowel capsule endoscopy to investigate possible complications in patients with non-responsive coeliac disease is evolving. Small bowel capsule endoscopy is a valuable diagnostic instrument for patients with obscure gastrointestinal bleeding and/or suspected small bowel tumors; it may also be a key examination in patients with suspected Crohn's disease, or patients with known Crohn's disease to fully assess disease extension and activity; finally, it may contribute for the diagnosis of complications of non-responsive coeliac disease.

Abstracts and Case Studies From the College of American Pathologists 2014 Annual Meeting (CAP '14)

Abstracts and Case Studies From the College of American Pathologists 2014 Annual Meeting (CAP '14)

Spectrum of Gastroenteropancreatic NENs in Routine Histological Examinations of Bioptic and Surgical Specimen: A Study of 161 Cases Collected from 17 Departments of Pathology in the Czech Republic

Objective. To characterize GEP-NENs in routine biopsies and surgical specimen in the Czech Republic and to evaluate how WHO Classification (2010) is acceptable in diagnostic practice. Methods. Paraffin-embedded blocks and bioptic reports were collected from 17 departments of pathology. Histologic slides were stained with H&E and immunohistologically for CgA, synaptophysin, and Ki-67. Results. Out of 28 gastric NENs, there were 22 NETs, G1, 5 NETs, G2, and 1 NEC. Ten duodenal NENs were NETs, G1. Among 27 NENs of jejunum and ileum, 23 were NETs, G1, 2 NETs, G2, and 1 NEC and 1 mixed adenoneuroendocrine carcinoma (MANEC). Among 42 appendiceal “incidentalomas”, 39 were NETs G1, 2 goblet cell carcinoids, and 1 MANEC. Out of 34 large intestinal NENs, 30 were NETs, G1, 3 NETs, G2, and 1 NEC. One small intestinal and 6 large bowel neoplasms were reclassified as poorly differentiated adenocarcinomas. In 12 pancreatic NENs, there were 7 NETs, G1, 3 NETs, G2, and 2 NECs. Conclusions. Our study demonstrates differences in GEP-NENs frequency in sites of origin in our region, comparing to other countries. Regarding routine bioptic diagnostics, we gave evidence that the WHO 2010 classification of NENs is fully acceptable for exact categorisation of tumours.

Pathologic distribution of disease at the time of interval debulking versus primary tumor reduction in women with primary peritoneal, ovarian, or Fallopian tube carcinoma.

5584 Background: The surgical approach for interval debulking after neoadjuvant chemotherapy (NACT) for primary peritoneal, ovarian, or fallopian tube carcinoma has largely been extrapolated from experience with primary tumor reductive surgery (PTRS). It is unknown whether procedures considered mandatory in PTRS, such as hysterectomy, contribute to comparable removal of macroscopic disease in the NACT setting. Our study compared the difference in pathologic distribution of disease at interval debulking surgery versus primary tumor reduction. Methods: After IRB approval, patients who received NACT or PTRS were identified through the tumor registry and surgical database at a single institution from 2008-2012. Involvement of organs at the time of surgery was categorized as either macroscopic, microscopic and no tumor. Statistical analyses included Wilcoxon Mann-Whitney and Fisher’s exact tests. Results: Of the 163 patients identified, 111 (67%) received NACT and 54 (33%) underwent PTRS. Median age was 62 and the majority of patients had stage IIIC high-grade serous carcinoma (91%). Macroscopic ovarian involvement was more common at time of PTRS (92 % vs 63 %, p &lt;0.001). Gross uterine involvement was significantly less in the NACT group compared to PTRS, with the majority of specimens in the NACT group free of disease (Macroscopic 11 % vs 42%, no tumor 62% vs 44 %, p &lt;0.002). However, 27% of the NACT had microscopic uterine serosal disease. Macroscopic large bowel involvement was 50 % in the PTRS vs 26 % in NACT(p&lt;0.005). There was no difference in disease involvement of the small bowel or omentum. Conclusions: The pathologic disease distribution at the time of interval tumor debulking is significantly different from that encountered during primary cytoreductive surgery.. NACT appears to reduce macroscopic large bowel and uterine tumor involvement and may negate the need for hysterectomy and/or large bowel resection at the time of interval debulking to achieve no gross residual.

Chagasic megacolon and large bowel neoplasms: case series and literature review

There is a clear association between chagasic megaesophagus and the esophageal cancer. On the other hand, the association between chagasic megacolon and intestinal neoplasm is uncommon. There are only a few cases described in literature. We selected two cases of colorectal adenocarcinoma associated with adenoma from 2000 to 2011, which are added to the four patients already described by this group. The mean age of the patients, was 68.5 years. Both had been submitted to surgical resection of the neoplasm. Survival rates ranged and were directly related to tumor staging at the time of diagnosis. In this context, we report our case series and reviwed the corresponding literature, especially the clinical and epidemiological aspects of this rare association.

Hyperplastic Polyposis Associated with Two Synchronous Colon Cancers

Hyperplastic polyps are common large-bowel tumors, are frequently detected in middle- to old- aged people, and usually are minuscule lesions in the distal colon and rectum. Most hyperplastic polyps have no malignant potential, but recent studies suggest that some hyperplastic polyps can progress to colorectal cancers, possibly by the so-called serrated pathway. Hyperplastic polyposis is a rare syndrome characterized by multiple hyperplastic polyps, primarily in the proximal colon. Different from sporadic hyperplastic polyps, hyperplastic polyposis is alleged to have high potential for malignancy because patients with this syndrome may frequently have conventional adenomas, serrated adenomas, and adenocarcinomas. We report the case of a patient with hyperplastic polyposis, who had two synchronous colon cancers, as well as sessile serrated adenomas and tubular adenomas.

Non-Hodgkin lymphoma of the descending colon in a child with obstruction; a case report and review of literature

Primary gastrointestinal non-Hodgkin lymphoma (NHL) accounts for 13-18% of all malignant tumours of small bowel and only 1% of large bowel tumours. The most common site of non-Hodgkin lymphoma of the intestinal tract in children is the terminal ileum and the ileocecal region. The non-Hodgkin lymphoma (NHL) of descending colon is extremely rare in children .We report non-Hodgkin lymphoma of the descending colon in an 8-year-old child with features of large bowel obstruction due to a large intraluminal single polyp. The diagnosis revealed non-Hodgkin lymphoma. Early diagnosis is mandatory and multimodality treatment is required to prevent complications and prolong survival.

Avaliação da expressão da caspase-3 no adenocarcinoma colorretal através de um sistema computadorizado de análise de imagem

RACIONAL: O câncer colorretal é uma das causas mais comum de mortes por neoplasias malignas no mundo ocidental, sendo que o adenocarcinoma colorretal representa mais de 90% dos tumores malignos que acometem o intestino grosso. A cisteíno-protease caspase-3, uma das mais participantes enzimas do processo apoptótico, pode ser utilizado como marcador tumoral. OBJETIVO: Identificar e quantificar a expressão da caspase-3 em amostras de adenocarcinomas colorretais através de um sistema computadorizado de análise de imagem, bem como correlacionar a expressão deste marcador. MÉTODOS: Foi estudada por imunoistoquímica a proteína caspase-3 em 55 amostras de adenocarcinoma colorretal parafinizadas. Para analisar os tecidos imunomarcados, o sistema SAMBA 4000 foi utilizado. Avaliaram-se as lâminas com o programa IMMUNO 4.00 através da leitura de 8 a 10 campos, determinando-se dois parâmetros: índice de marcagem (LI, Labeling Index) que descreve a porcentagem de área tecidual especificamente marcada pela prova imunoistoquímica, e a densidade óptica média (MOD, Mean Optical Density), a qual representa a intensidade de coloração do tecido medida por pixels positivos. Para a análise estatística, utilizou-se o teste t-Student e o coeficiente de Pearson para verificar a existência de correlação entre índice de marcagem e a densidade óptica média do mesmo marcador. RESULTADOS: Todas as amostras estudadas apresentaram positividade à caspase-3 (LI médio: 85,24% ± 8,71% de células positivas). Houve correlação estatisticamente significante entre o índice de marcagem e a densidade óptica média deste marcador. CONCLUSÃO: A intensidade da expressão de caspase-3 e o número de células expressando este marcador correlacionam-se positiva e significantemente.

Survival From Malignant Digestive Endocrine Tumors in England and Wales: A Population-Based Study

Little is known about the prognosis of patients with malignant digestive endocrine tumors (MDETs), primarily because of their rarity. Survival from these tumors has been evaluated in a large, well-defined, national population. All patients diagnosed and registered in England and Wales during the 14-year period from 1986 to 1999 were followed up for vital status to the end of 2001. Relative survival was estimated and the impact of age, sex, period, histology, and anatomic site modeled. Among 4104 cases of MDETs, 21.2% were small cell tumors. Relative survival for all MDETs combined was 45.9% at 5 years and 38.4% at 10 years. Five-year survival was 56.8% for well-differentiated tumors but only 5.2% for small cell tumors (P < .0001). Survival was highest for large bowel tumors and lowest for esophageal tumors. Among well-differentiated pancreatic tumors, 5-year relative survival was 49.2% for insulinomas, 39.9% for gastrinomas, 17.1% for glucagonomas, 26.3% for carcinoid tumors, and 29.3% for nonfunctioning tumors. There was no difference in survival between socioeconomic groups. Five-year survival did not improve between 1986 and 2001. Survival was higher for women and for younger patients. Gender, age at diagnosis, and anatomic site were independent prognostic factors. The prognosis of patients with MDETs in the general population is considerably worse than is often reported from small hospital case series. Prognosis varies with tumor differentiation, anatomic site, and histologic type. Early diagnosis is difficult; new therapeutic options appear to represent the best approach to improved prognosis.