Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of 14-year-old girl is reported who presented with features of tuberculous subacute intestinal obstruction. Exploratory laparotomy revealed a urachal cyst associated with pseudomyxoma peritonei (PMP). Histopathology confirmed a moderately differentiated mucin secreting adenocarcinoma of urachal cyst associated with PMP. The adenocarcinoma of urachal cyst associated with PMP is further reviewed.
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PMID:Adenocarcinoma of urachal cyst associated with pseudomyxoma peritonei masquerading as abdominal tuberculosis: A case report and review of literature. 1946 8

Intussusception is a rare cause of intestinal obstruction in adult patients, but is common in children. In fact, it accounts for an estimated 1% of all cases of bowel obstruction in adults, although adult intussusception of the large intestine is rare. Sigmoidorectal intussusception, however, is a rare variety with few cases reported in the literature. A mucinous adenocarcinoma, a subtype of adenocarcinoma, is characterized by extracellular mucin production and accounts for between 5% and 15% of the neoplasms of the colon and rectum. Despite the general consensus supporting surgical resections for adult intussuceptions, controversy remains over whether intussuceptions should be reduced before resection. Most cases of colon intussusception should not be reduced before resection because they most likely represent a primary adenocarcinoma. However, prior reduction followed by a resection can be considered for the sigmoidorectal intussusception to avoid inadvertent low rectal cancer sugery. We experienced one case of sigmoidorectal intussusception caused by a mucinous adenocarcinoma of the sigmoid colon in a 79-year-old woman. Abdominal computed tomography demonstrated a sigmoidorectal intussusception. After the end-to-end anastomosis-dilator-assisted reduction, the patient underwent a laparoscopic oncological anterior resection under the impression that a sigmoidorectal intussusception existed. We report a successful laparoscopic anterior resection in a patient with an intussusception caused by a sigmoid malignant tumor.
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PMID:Laparoscopic treatment of adult sigmoidorectal intussusception caused by a mucinous adenocarcinoma of the sigmoid colon: a case report. 2143 Oct 97

Colorectal carcinoma is very rare in childhood. In this case report, we depict a ten-year-old girl who presented with features of intestinal obstruction which turned out to be due to poorly differentiated mucin secreting adenocarcinoma of descending colon. Only increased awareness of this malignancy in this age-group and a high index of suspicion can help when a child complains of persistent pain of abdomen, altered bowel habits or rectal bleeding, and may provide diagnosis at an earlier stage, thereby improving the prognosis.
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PMID:Colorectal carcinoma in a ten-year-old girl: a case report. 2253 29

Goblet cell carcinoid (GCC) is a rare variant of carcinoid tumor that exclusively involves the appendix. It usually occurs in 5th-6th decade with the most common clinical presentation being acute appendicitis. The natural history of this tumor is intermediate between carcinoids and adenocarcinomas. We here report a case of GCC diagnosed incidentally in a patient presenting with acute intestinal obstruction. Ultrasonographic examination supported the clinical diagnosis of acute intestinal obstruction, following which the patient underwent laparotomy and resection of ileum along with appendix was done. On gross pathological examination, a nodular growth was present on the tip and body of appendix that was yellow in color with a semi-solid to mucoid consistency on cut section. On microscopy, lakes of mucin with few acinar structures floating in them were seen. The submucosa as well as serosa were infiltrated by clusters of goblet cells and well-formed acini, with little atypia. Glands and nests were positive for periodic acid Schiff and immunohistochemistry showed focal chromogranin positivity in glandular structures, thereby confirming the diagnosis of GCC. Although the prognosis of GCC is better than adenocarcinomas, it is one of the carcinoids having a poorer outcome when compared with other variants of carcinoid tumor. Therefore, it is important to rule out other differential diagnoses of goblet cell carcinoid, the most important being mucinous adenocarcinomas.
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PMID:Incidentally discovered goblet cell carcinoid clinically presenting as acute intestinal obstruction: a case report with review of literature. 2473 49

Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1-2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article.
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PMID:Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects. 2488 59

Diffuse malignant peritoneal mesotheliomas in children are uncommon, aggressive tumors with a grave prognosis. We herein report the clinical, radiologic, and pathologic findings of a 16-year-old male. The adolescent presented with a history of abdominal pain, nausea and daily, nonbilious, nonbloody emesis for 3 weeks. Radiographic imaging suggested small bowel obstruction. The diagnostic work-up and differential diagnoses are discussed. Histologically, the tumor was composed of epithelioid cells with a papillary and glandular architectural pattern. A few glands appeared to produce mucinous material. Histochemistry revealed PAS diastase resistant mucin, an inconspicuous finding in diffuse malignant peritoneal mesothelioma. An extensive immunohistochemistry panel (calretinin, WT-1, D2-40, CK 7, CAM 5.2, CK 5/6, CEA, B72.3, CK 20, CD10, CD30, CD15, CD117, PLAP, S100, TFE3, and EMA) confirmed the diagnosis. Of special interest, BAP1 staining was cytoplasmic and consistent with 3p deletion detected by conventional cytogenetics. The ultrastructural analysis demonstrated long microvilli, desmosomes, and intercellular junctions which further supported the diagnosis.
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PMID:Malignant Peritoneal Mesothelioma in an Adolescent Male With BAP1 Deletion. 2522 65

Pseudomyxoma peritonei (PMP) is an uncommon clinical condition that typically originates from a perforated epithelial neoplasm of the appendix. The clinical presentation is variable, often with non-specific symptoms and is associated with abdominal distension in advanced cases. Whilst traditionally considered benign, it is apparent that PMP represents a spectrum of disease and, at best, should be considered a "border-line" malignancy. The condition is characterised by the development of mucinous ascites. Tumour cells and mucin accumulate at characteristic sites within the peritoneal cavity according to the redistribution phenomenon, usually sparing the mobile small bowel. In advanced cases, high volume disease and mucinous ascites lead to compression of the gastrointestinal tract, bowel obstruction, and ultimately, starvation. Controversy still exists over the pathological classification of PMP and its prognostic value. Computed tomography remains the optimal preoperative staging investigation. Elevation of serum tumour markers correlates with a worse prognosis. Optimal treatment involves cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC). With complete cytoreduction and HIPEC an 80% 5 year survival can be achieved in patients with low grade disease. Maximal tumour debulking can produce good palliation and long term survival in a small number of patients. Initial high morbidity and mortality is seen to decrease with increasing experience and this is likely to represent improvement in patient selection and postoperative management as well as surgical expertise.
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PMID:Management of pseudomyxoma peritonei. 2605 32

Pseudomyxoma peritonei is characterized by mucinous ascites originating from a mucin-producing neoplasm; however, even the definition is still under debate. Tumor deposits extend and ultimately engulf the entire cavity, causing death from cachexia due to limited intestinal movement. Here, we report a unique case of an 80-year-old woman with pseudomyxoma peritonei, which extended to the lower extremity mimicking infectious condition. The patient survived for a long time without bowel obstruction despite having the histologic subtype that has an unfavorable prognosis. The extremity lesion was treated with limited extensive surgery. The origin of the disease and the mechanism of extension to the extremity could not be clarified. Clinicians should be aware of the original disease entity and this unusual presentation and determine its mechanism and the best management strategy.
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PMID:Pseudomyxoma peritonei extending to the lower extremity: a case report. 2618 69

Signet-ring cell carcinoma (SRCC) is an adenocarcinoma characterized by mucin-producing cells and most commonly arises in the stomach. Colonic SRCC can share features of colitis, including long segments of concentric bowel wall thickening and ulcerated mucosa with regions of sparing. We describe a rare case of metastatic gastric SRCC mimicking Crohn's disease. Our patient underwent 2 colonoscopies, and biopsies revealed chronic active inflammation with no evidence of malignancy. The diagnosis of SRCC was only made after colectomy was performed for recurrent bowel obstruction.
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PMID:Metastatic Gastric Signet Ring Cell Carcinoma Mimicking Crohn's Disease. 2977 26

Mucinous neoplasm of the appendix are rare entities, among these, mucinous cystadenomas contribute to 31-34%. Cystadenomas often produce extensive dilatation of the appendix with epithelial atypia. Spontaneous perforation often occurs in 20%, leading to mucin distribution throughout the peritoneal cavity. Half of the patients are completely asymptomatic and are detected as an incidental diagnosis, others symptoms include a palpable mass, hernias, weight loss, peritonism or even intestinal obstruction. We present a case of a 71-year-old female, she presented with a palpable mass in the upper abdomen. A ventral hernia was the most likely diagnosis and hernioplasty was planned. At surgery, a mucinous mass was discovered and mucous material was found free en the peritoneal cavity. Also, a perforated appendix and a mass in the cecum was found. Patient underwent full recovery. Pathology reported pseudomyxoma peritonei as the final diagnosis.
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PMID:Abdominal hernia and the unexpected final diagnosis. 2997 5


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