Appendiceal Mucinous Neoplasms: Diagnosis and Management.

OBJECTIVE: Appendiceal mucinous neoplasms (AMNs) are a rare and heterogeneous disease for which clinical management is challenging. We aim to review the literature regarding modalities of treatment to guide the management of AMNs. METHODS AND REVIEW CRITERIA: We conducted a PubMed search in February 2016 for English-language publications, using the terms "appendiceal," "appendix," "carcinoma," "cancer," "mucinous," "treatment," "genes," "target," "genomic," and terms listed in the articles' subheadings. Published reports and abstracts from the American Society of Clinical Oncology meetings were also searched. RESULTS: In this review, we summarize current data and controversies in AMN classification, clinical presentation, molecular alterations, treatment outcomes with regard to cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), and the role of systemic chemotherapy. CONCLUSION: Appendiceal mucinous neoplasms are a heterogeneous group of tumors with a rising incidence. Treatment is based on stage and histology. Low-grade tumors are treated surgically with resection of the primary site in early stage disease, or peritoneal debulking and HIPEC in patients with advanced stage disease. Treatment of high-grade tumors requires further prospective trials, and options include debulking surgery and HIPEC with or without preoperative chemotherapy. Trials evaluating novel therapies based on the molecular profiling of AMN tumors are needed to evaluate therapeutic options in patients who are not surgical candidates. IMPLICATIONS FOR PRACTICE: This review provides a reference to guide gastroenterologists, pathologists, surgeons, and oncologists in the management of appendiceal mucinous neoplasms (AMNs), a rare and heterogeneous disease with no consensus on histologic classification or guidelines for treatment algorithms. This review summarizes all AMN classifications and proposes a treatment algorithm based on stage and histology of disease.

Synchronous quintuple primary gastrointestinal tract malignancies: Case report.

Multiple primary malignancy is defined as two or more malignancies detected in an individual person. In particular, synchronous quintuple primary malignancy is extremely rare. A 52-year-old male with anal pain and intermittent blood-tinged stool was diagnosed with malignancies in the stomach, jejunum, ascending colon, transverse colon and rectum. He underwent a subtotal gastrectomy, segmental resection of the jejunum and total protocolectomy with end ileostomy. The postoperative pathologic findings were moderate differentiated gastric adenocarcinoma (pT1bN0M0, pStageIA), combined adenocarcinoma and neuroendocrine carcinoma of the jejunum (pT3N0M0, pStageIIA), three mucinous adenocarcinoma of the ascending colon (pT3N0M0, pStageIIA), transverse colon (pT1N0M0, pStageI) and rectum (pT3N1aM0, pStageIIIB). The tumors did not lack MLH-1 and MSH-2 expression, as the markers (bat26, D5S346, bat25, D2S123) suggest MSI-H presence. Adjuvant chemoradiotherapy was started according to regimen, FOLFOX 4 for advanced rectal cancer. Six years post-operation, the patient is currently attending regular follow-ups without recurrence or metastasis.

Early identification of DPAM in at-risk low-grade appendiceal mucinous neoplasm patients: a new approach to surveillance for peritoneal metastasis.

BACKGROUND: Disseminated peritoneal adenomucinosis (DPAM) patients often have a history of appendectomy with identification of an incidental mucinous neoplasm (low-grade appendiceal mucinous neoplasm (LAMN)). The rate of developing DPAM is not well established. METHODS: Twenty-two patients with incidental LAMN were identified and monitored with cancer markers and CT every 4-6 months. Laparoscopy with peritoneal washing was performed in patients either in the event of radiographic disease or after 12 months in absence of radiographic disease. The rate of detecting peritoneal metastasis was determined for CT scan and laparoscopy. RESULTS: Peritoneal metastasis was detected in 5 (23 %) patients. Occult disease was detected in four patients at laparoscopy without a detectable disease on CT scan. One patient developed radiographic progression at 6 months confirmed with laparoscopy. Four patients were treated with cytoreductive surgery (CRS)/HIPEC and one with CRS only. The 17 patients with negative laparoscopy remain disease free with a median follow-up of 50 months. CONCLUSIONS: The rate of peritoneal metastasis in incidental LAMN patients was 23 %. Laparoscopy was the primary screening tool identifying occult metastasis. The median PCI of 7 was low, and all the patients underwent R0/R1 resections. This study revealed 1 in every 4.4 patients with LAMN may develop PMP. Longer follow-up and further patient surveillance is warranted.

The Diagnostic Significance of a Mucocele of the Appendix.

Appendiceal mucoceles are rare lesions with a variable clinical presentation often identified incidentally on imaging or at laparotomy/laparoscopy for an unrelated diagnosis. Mucocele of the appendix may be a benign or malignant process, making early recognition based on symptoms and key radiographic characteristics of the utmost importance for optimal patient management. Here we present the case of a patient presenting with non-specific abdominal complaints suffering from appendiceal mucocele perforation due to low-grade mucinous adenocarcinoma.

[Clinical analyses of 70 cases of multiple primary colorectal carcinoma].

BACKGROUND & OBJECTIVE: Multiple primary colorectal carcinoma (MPCC) is not rarely seen, but it possesses a unique biological characters. This study was to investigate the clinical characteristics, diagnosis, therapeutic principle and prognosis of MPCC. METHODS: Data of 70 MPCC patients, treated by operation from 1997 to 2003, were analyzed. Of the 70 patients, 61 had synchronous carcinoma (SC) and 9 had metachronous carcinoma (MC). RESULTS: Fifty-five patients were diagnosed by colonoscopy, barium enema or CT scan pre-operationally, while 15 were diagnosed intra-operationally due to the oversized tumor at the distal end of the colon. Thirty-three patients had colorectal carcinoma accompanying with adenoma and multiple polyps. All the patients underwent surgical resection except 3, who received short-circuit operation because of unresectable lesions. Fifty-two patients received radical resection, while 15 received palliative resection due to hepatic or peritoneal metastasis. The overall 3-and 5-year survival rates were 65.7% and 45.7%. In the patients who received radical resection, the 3-and 5-year survival rates were 78.1% and 59.3%. CONCLUSIONS: The occurrence of MPCC is largely related with adenomas and polyps. The extent of resection should be individually determined by the lesion location, range, the distance of lesions as well as the general condition of the patients. Prognosis of MPCC is relatively good. The patients accompanying with adenoma and multiple polyps should be followed up intensively.

Raised serum chondroitin sulfate epitope level in ovarian epithelial cancer.

OBJECTIVE: To determine the value of serum chondroitin sulfate epitope WF6 and hyaluronan (HA) levels as a biomarker for early detection of ovarian epithelial cancer and other gynecological disorders. METHOD: Serum WF6 CS epitope and HA were measured in 91 patients with ovarian epithelial cancer, 39 patients with non-cancer gynecological disorders and 30 healthy women. Serum chondroitin sulfate (CS) WF6 epitope was determined by a competitive immunoassay with the monoclonal antibodies WF6, which specifically recognizes an epitope in native CS chains. In addition, serum HA concentration was measured by an ELISA-based assay with a biotinylated affinity HA-binding proteins. RESULTS: The serum concentration of CS (WF6) epitope was highly increased in epithelial types of ovarian cancer and at all stages of development (p < 0.005). Serum HA in ovarian cancer patients was significantly higher than normal controls (p < 0.05). CONCLUSION: These results reflect changes in ECM metabolism in progressive ovarian cancer, which cause an increase in serum CS epitopes and HA. Therefore, serum CS epitopes may provide useful biomarkers for cancers and other disorders of the ovary. Measurement of serum HA provided complementary information, which may be useful as a discriminator between benign ovarian disorders and malignant ovarian diseases.

Cáncer del uraco / Urachal cancer

Se presenta el caso clínico de un hombre de 75 años que consulta por baja de peso, dolor, masa infraumbilical asociado a constipación y dificultad para orinar, de 12 meses de evolución. Se estudió con ECO, TAC, enema baritado, colonoscopia y cistoscopia. Se efectuó resección en bloque de un tumor uracal con pared abdominal infraumbilical comprometida, onfalectomía, asa de íleon adherida, rectosigmoides y lecho vesical, reconstituyendo inmediatamente el tránsito con anastomosis ileoileal y colorrectal. El estudio histológico reveló un adenocarcinoma mucinoso del uraco. Se clasificó en estadio III. El paciente presenta buena evolución, sin recidiva a los 6 meses del postoperatorio, corroborada con endoscopia y TAC. El cáncer del uraco es un cuadro rarísimo, habiéndose comunicado alrededor de 450 casos en la literatura. Su tratamiento es esencialmente quirúrgico y el pronóstico a largo plazo es pobre, con una sobrevida a 5 años entre 6 a 50 por ciento

[Appendiceal mucinous carcinoma]. / Rak sluzowy wyrostka robaczkowego.

Appendiceal carcinoma is very rare. Preoperative diagnosis of this kind of neoplasm is difficult. Even at operation in under half of cases this diagnosis is considered. We present a case of a woman admitted to the hospital with diagnosis of adnexal tumor of the right ovary. The diagnosis of adnexal mass was confirmed by ultrasound examination. Laparotomy was performed and tumor of appendix was found. There was also a small tumor (2 cm) in small bowel and some increased lymphatic nodules. Uterus with adnexis, tumor of appendix, part of small bowel and colon were resected. Because of infiltration of the neoplasm a part of the bladder was resected as well. There were no complications after operation. She received chemotherapy with 5-FU. After 9 months computed tomography revealed no changes in abdomen. Diagnosis of pelvic masses when they do not origin of adnexis is usually very difficult. In many cases proper diagnosis is made only during laparotomy.

Colorectal carcinoma in children.

BACKGROUND/PURPOSE: Colorecta I carcinoma is extremely rare in children and presents with a poor prognosis. Surgical management and long-term follow-up of this entity are still obscure because of lack of data. Therefore, a retrospective clinical trial was performed to evaluate the clinical characteristics of childhood colorectal carcinoma and to determine the predictors of poor outcome. METHODS: Records of children who had colorectal carcinoma and were treated at our unit between 1972 and 1997, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, clinical characteristics, diagnostic procedures, extent of disease, treatment methods, histological types, and outcome. A modified Dukes staging scheme was used. RESULTS: There were 12 boys and 8 girls who were treated for colorectal carcinoma (range, 7 to 16 years). Predisposing conditions were encountered in 2 patients (10%), one with Bloom's syndrome and another with familial occurrence of colonic carcinomas. Predominant symptoms were abdominal pain, vomiting, and rectal bleeding. Barium enema, ultrasonography, computerized tomography, and endoscopic procedures were used for the diagnosis. Rectosigmoid region was the most common site for the primary tumor (65%). All patients presented with advanced stages of disease (stage C, 7; stage D, 13). Surgical procedures were incisional biopsy (n = 4), palliative permanent colostomy (n = 4), segmental resection (n = 5), complete resection (n = 6), and rectal biopsy (n = 1). Peritoneum was the most common site of extensive intraabdominal disease followed by omentum majus and liver. The lung also was involved in 2 patients. Predominant histological type was mucinous adenocarcinoma (80%). All patients but one received adjuvant chemotherapy, and 2 received palliative radiotherapy. Thirteen patients died of disease in a period ranging from 1 day to 1 year after initial surgery. The fate of 4 patients who were discharged in a very ill status was unknown. Three patients were alive for 2 years to 4 years postoperatively, and one of them presented with end stage disease. CONCLUSIONS: Delayed diagnosis, advanced stages of disease at presentation, and, most importantly, mucinous type of histology are the major determinants of poor outcome in childhood colorectal carcinoma. We emphasize that possibility of a malignant colorectal tumor should be considered for any childhood case with signs and symptoms of intestinal obstruction, intractable abdominal pain, alteration in bowel habits and gastrointestinal bleeding. Colorectal malignancy should not be excluded only on the basis of the patient's age. Because of the steadily increasing incidence of precancerous bowel diseases and poor prognosis of colorectal carcinoma, childhood cases of bowel disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment as given to adult cases. Contrast studies, ultrasonography, computed tomography, and endoscopy are essential procedures for both confirming the diagnosis and detecting the extent of the disease.

Adult colonic intussusception caused by malignant tumor of the transverse colon.

Adult colonic intussusception is rare and often originates from neoplasms. In emergency situations it can be difficult to diagnose. Our aim was to show how the integration of readily available diagnostic means in emergency situations can help in making a correct diagnosis of this disease. A 68-year old male patient presented with vomiting and abdominal pain. The abdomen was distended, with pain to palpation in the left quadrants without a mass. Plain radiographs of the abdomen showed a large amount of gas in the small bowel and in the right and transverse colon. A barium enema demonstrated an endoluminal filling defect in the descending colon. Abdominal ultrasonography revealed the presence of intraperitoneal fluid and thickened left colonic wall at the site of the lesion, with an aspect of a "double ring" consistent with intussusception. A solid formation was also revealed at a point distal to the thickened colonic wall. At emergency laparotomy an approximately 8-cm-long mass was palpable through the left colon. A colostomy was fashioned, and subsequently colonoscopy revealed the presence of a left colon tumor. At the subsequent operation an invagination of the left transverse colon into the descending colon was confirmed. The left transverse and descending colon were resected with high ligation of the left colic artery. Macroscopic examination of the invaginating head showed a vegetating transverse colon neoplasm. We conclude that in emergency settings the association of readily available diagnostic means such as plain abdominal radiography, water soluble contrast enema and ultrasonography may yield reliable information for diagnosing colonic intussusception.

[Colorectal cancer: study on 365 cases]. / Cáncer colorectal: estudio sobre 365 casos.

We report a clinic-endoscopical study about 365 patients, both of sex, between 26-95 years old, with colonoscopic diagnosis of colorectal cancer. Results showed that 61,92% were men and 38,08% women; in 92,60% the disease ocurred over 40 years old. 13,42% had malignant personal history -colorectal cancer, uterus and breast cancer, and others-; 13,97% had bening personal history-colorectal adenoma, cholecystectomy, and others-; Abdomina pain, change in intestinal habits, and bleeding were the moist frequent symptoms, with differences depending of the tumors localization in the colon or rectum. 62,57% of patients had anemia under 10g% of hernoglobin; in 85,23% the fecal occult blood test was positive. On 199 patients, the simple barium enema diagnosed the tumor in 66,33% only; but in the same group, colonoscopy diagnosed the cancer in 96,49% at first examination. In all patients, colonoscopywas excellent for diagnosis of the principal lesion, and for the identification of synchronous neoplasia. On 365 patients, colonoscopy diagnosed the cancer in 98,08% at first examination. The localization of tumors was: 57,63% in left colon (49,47% in rectum and sigmoid colon); 34,21% in the right colon; and 8,16% in transverse. Pathology showed that adenocarcinoma was the most frequent tumor 95,23%; 1,06% mucoid carcinoma; 1,06% epidermoid carcinoma; and 2,65% lymphorna. In 32,05% of cases there were synchronous lesions; 3,01% had other cancer, and 54 patients had 112 polyps (62,50% adenomatous polyp, 6,25% adenoma with non invasive or invasive adenocarcinoma, and 31,25% hiperplastic polyp. Authors emphasize the value of the detection and early diagnosis to decrese the colorectal cancer mortality.

The unusual presentation of mucinous carcinoma in the colon making preoperative diagnosis difficult: report of a case.

A 51-year-old man was admitted to our hospital for investigation of fever, lower abdominal pain, and pain on micturition. Barium enema and endoscopic findings revealed an extracanal large cavity of the rectosigmoid colon, which suggested an abscess caused by penetration. Histological examination of the endoscopic biopsy specimen demonstrated no malignancy. A preoperative diagnosis of malignant lymphoma was strongly suspected from the imaging findings; however, intraoperative inspection revealed a large tumor invading the urinary bladder. An intraoperative incisional biopsy was performed and histological examination demonstrated mucinous carcinoma. The patient subsequently underwent pelvic exenteration about 1 week later followed by adjuvant chemotherapy and radiotherapy.

Synchronous colorectal carcinomas.

Eighteen (5.0%) out of 358 patients who underwent resection of a colorectal carcinoma during the period 1978 through 1990 had synchronous colorectal carcinomas, and were 5.6 years younger on average than those with a single carcinoma. The distance between synchronous lesions was less than 10 cm in 69.6% of all the patients in the study. Among the synchronous carcinomas there was a higher incidence of ascending colon involvement, mucinous carcinoma, family history of malignant diseases, multiple malignant neoplasms associated with other organs and benign neoplastic polyps of the colorectum, and it is suggested that hereditary oncogenic factors influence these carcinomas. The synchronous lesions were detected pre-operatively in 14 of 18 patients with synchronous carcinomas, and the most common reason why synchronous lesions were missed was that the lesions on the anal side prevented the lesions on the proximal side from being examined. The prognosis in the synchronous lesion group was worse than in the solitary lesion group. Since it is difficult to predict synchronous colorectal carcinomas, careful pre-operative examination, including that of other organs, is necessary, and intra-operative colonoscopy should be carried out when pre-operative examination was insufficient.

Human breast cancer in vivo: H-1 and P-31 MR spectroscopy at 1.5 T.

To assess the potential of in vivo magnetic resonance (MR) spectroscopy for breast cancer, hydrogen-1 and phosphorus-31 MR spectra of five malignant human breast tumors were compared with those of unaffected breast tissue. The water-to-fat ratio was high in the tumors (average, 2.2) but low in the unaffected tissue (average, 0.3). The P-31 spectrum of normal breast tissue showed low levels of phosphomonoesters (PMEs), inorganic phosphate, phosphodiesters (PDEs), and ATP. In addition, an intense phosphocreatine (PCr) signal was observed in breast tissue of young women: The relative intensities of the PCr and ATP signals had a mean value of 1.9. The tumor spectrum showed elevated levels of PMEs, Pi, and PDEs, while no PCr was seen (PCr/ATP less than 0.2). In two breast cancers treated with radiation therapy, resulting in a decrease of tumor volume of more than 50%, a similar change in the tumor P-31 spectrum was observed: An intense PCr signal developed (PCr/ATP = 1.1). Control experiments indicated that the appearance of PCr after radiation therapy was the result of a radiation-induced metabolic change in the tumor itself.

Carcinoma of the colon: diagnosis by ultrasound and enema.

Carcinoma of the colon presenting before puberty is rare and few cases have been recorded in radiological literature. The symptomatology is usually vague but may be similar to the classical presentation of adults. The barium enema will also show the same constricting lesions. Two cases are reported, in one of which the initial diagnosis was made by ultrasound.