Primary linitis plastica of the rectum. Report of two cases.
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BACKGROUND: Dissemination of cancer cells in the abdominal cavity may lead to peritoneal carcinomatosis, a common event in the linitis plastica type of gastric carcinoma. Reverse transcriptase polymerase chain reaction (RT-PCR) is a sensitive technique for detecting these cells. METHODS: Peritoneal washings were obtained during laparotomy from 47 consecutive linitis plastica patients who preoperatively were considered candidates for curative resection. Together with conventional cytological examination using Papanicolaou staining, real-time RT-PCR was performed to quantitate prospectively carcinoembryonic antigen (CEA) mRNA in these washings. Samples above a cutoff value for CEA mRNA were considered positive for molecular detection of disseminated cancer cells. RESULTS: Conventional cytological examination was positive in 43% (20/47). Positivity of CEA mRNA was much higher at 83% (39/47) and peritoneal carcinomatosis actually was observed in 77% (36/47), either at laparotomy or during postoperative follow-up. Although only two of eight patients who were negative for CEA mRNA suffered from peritoneal carcinomatosis, three more patients died of recurrences through other metastatic pathways. Multivariate analysis revealed that curability of the operation (knowledge of the CEA mRNA values excluded) and T categories were the only significant independent prognostic factors. CONCLUSIONS: RT-PCR of the peritoneal washes had little value as a prognostic factor, but identified over 80% of the patients planned for curative surgery to have disseminated cancer cells in the peritoneal cavity. Almost the same proportion of patients actually suffered from peritoneal carcinomatosis. These facts indicate why treatment with surgery alone rarely cures patients with linitis plastica.
Two cases of signet-ring cell adenocarcinoma of the urinary bladder with a linitis plastica pattern of infiltration were studied. Mucin histochemistry indicated the presence of neutral and acid mucopolysaccharides and absence of sulphomucin. Immunocytochemistry of the tumour cells was positive for keratin and carcinoembryonic antigen, but negative for prostatic specific antigen and vimentin. The relevance of these observations to the differentiation and histogenesis of these tumours is discussed.
Of eleven cases of adenocarcinoma of the appendix that were studied, five were examples of mucinous cystadenocarcinoma and four were examples of colonic-type adenocarcinoma. Two lesions were very distinctive, with thick, cord-like transformation of the appendix. The term linitis plastica carcinoma is proposed to describe this rare variant of appendiceal carcinoma. Justification for the separation of linitis plastica carcinoma from the other two types is based on morphological appearance and on outcome. Both our linitis plastica patients died of metastases within six months of surgery, whereas, in the other two groups, death was directly related to spread of the carcinoma in only two of nine patients.
Intestinal cancer is uncommon in Crohn's disease but the risk of developing such a tumor is increased. Linitis plastica of the small bowel or colon is very rare. We report a case of ileocolonic linitis plastica which occurred 21 years after an ileocecal resection for Crohn's disease. Partial small bowel obstruction in relation with stricture of the preanastomotic loop prompted us to suspect disease recurrence. The tumor was not diagnosed either on preoperative work-up, or during surgery but only on the histological examination of the resected specimen. Palliative chemotherapy with 5 FU and folinic acid was performed. The patient was asymptomatic after a 17-month follow-up. This observation focuses on the clinical signs and course of linitis plastica. It also illustrates the difficulty of tumor diagnosis in Crohn's disease. Malignant transformation must be suspected if signs of active disease re-occur after a lengthy quiescent period.
PURPOSE: To evaluate the results of postoperative irradiation +/- chemotherapy for carcinoma of the stomach and gastroesophageal junction. METHODS AND MATERIALS: The records of 63 patients who underwent resection for stomach cancer were retrospectively reviewed. Twenty-five patients had complete resection with no residual disease but with high-risk factors for relapse. Twenty-eight had microscopic residual and 10 had gross residual disease. Doses of irradiation ranged from 39.6 to 59.4 Gy with a median dose of 50.4 Gy in 1.8 Gy fractions. Fifty-three of the 63 (84%) patients received 5-fluorouracil (5-FU)-based chemotherapy. RESULTS: The median duration of survival was 19.3 months for patients with no residual disease, 16.7 months for those with microscopic residual disease, and 9.2 months for those with gross residual disease (p = 0.01). The amount of residual disease also significantly impacted locoregional control (p = 0.04). Patients with linitis plastica did significantly worse in terms of survival, locoregional control, and distant control than those without linitis plastica. The use of 4 or more irradiation fields was associated with a significant decrease in the rate of Grade 4 or 5 toxicity when compared to the patients treated with 2 fields (p = 0.05). CONCLUSIONS: There was a significant association between survival and extent of residual disease after resection as well as the presence of linitis plastica. Distant failures are common and effective systemic therapy will be necessary to improve outcome. The toxicity of combined modality treatment appears to be reduced by using greater than 2 irradiation fields.
AIMS: The purpose of this study was to investigate the epithelial lesions associated with signet ring cell carcinoma (SRCC) of the colon and small intestine and the possible mechanism of tumour development. METHODS: Twenty-seven cases of adenocarcinoma with a signet ring cell (SRC) component of the colon and small intestine were divided into three groups depending on the association of the SRCC with: (1) epithelium without definite epithelial dysplasia, (2) adenoma, and (3) common type of adenocarcinoma (CTCA) with SRCC component occupying more than 50%, 30%, or less than 30% of the tumour. RESULTS: Most carcinomas were of T3 or T4 type, using the TNM standard staging system. The SRCC component was histopathologically similar in all groups. In group 1 (four cases, linitis plastica type), the overlying epithelium was normal or showed indefinite epithelial dysplasia and occasionally contained intra-epithelial SRCs. In groups 2 and 3 (two and 21 cases, respectively), seven cases contained multiple foci of intra-epithelial SRCs in areas separated from the invasive carcinoma. Transitional areas between SRCC and adenoma or CTCA were also identified. Immunostaining for p53 showed a varied extent of positive reactivity in 23 SRCC. The degree and the extent of reactivity appeared to increase with the stage of the carcinoma. Most intra-epithelial SRCs were immunoreactive for p53. Linitis plastica SRCC was associated with extensive p53 reactivity of the 'atypical' and the adjacent 'normal' epithelium. CONCLUSIONS: SRCC may arise from either CTCA, adenoma, 'atypical' epithelium or a combination of these epithelia. SRCC accounts for the bulk of carcinoma in each of these categories. In linitis plastica SRCC, positive reactivity for p53 is extensive in the adjacent 'normal' colonic epithelium and extends as far as 3cm from the microscopically identified SRCC margin.
Of 1192 patients treated for breast cancer, four had extrahepatic gastro-intestinal metastases as first clinical manifestation of the tumour dissemination. One woman presented with gastric metastases mimicking a linitis plastica. Another had metastases localized to the rectum also mimicking a linitis plastica. Two women had peritoneal and retroperitoneal metastases that caused, in one case, a right hydronephrosis. Histology of the four primary tumours showed invasive lobular carcinoma (ILC) mixed with invasive ductal carcinoma in two. However, ILC exclusively was found at the site of the gastro-intestinal metastases involving the serosal layer (two cases) and extending to the submucosa (one case) or to the mucosal stroma (one case). Thus, when a women with previous history of invasive lobular breast cancer experiences gastro-intestinal symptoms, particular attention should be paid to the large and deep biopsy of lesions to ascertain the histological type and whether oestrogen or progesterone receptors differ from those of the primary breast lesion. Since survival is extremely variable (one woman is alive 7 years after the discovery of gastro-intestinal metastases), treatment including surgery, hormonal manipulation and chemotherapy with the expectation of a cure is often justifiable, particularly if no other extensive metastases are present.
Locally advanced gastric adenocarcinomas (LAGC) have a poor prognosis, particularly when tumours are bulky, located in the cardia or in the event of locoregional lymph node involvement. Patients bearing these tumours were entered in a phase II trial of neoadjuvant chemotherapy, combining continuous intravenous 5-fluorouracil (5FU) (1000 mg/m2 for 5 days) and cisplatinum (CDDP) (100 mg/m2 on day 2) repeated every 4 weeks, for one to six cycles according to response and tolerance. 30 patients have been entered, 26 after clinical evaluation (CAT scan and upper gastrointestinal endoscopy) and 4 with unresectable tumours at prior laparotomy. Median age was 60 years, 15/30 patients had a tumour of the cardia, 15/30 had enlarged lymph nodes and 7/30 had linitis plastica (diffuse type). A mean number of three cycles was administered (range 1-6). 27 of the 30 patients were evaluable for response. One patient achieved a complete response (CR) and 14 a partial response (56%; 95% confidence interval 38-74%). No patient had tumour progression, and only 1/6 with linitis plastica responded. 28 patients underwent surgery, and 23 had a macroscopically complete resection (77% of the 30 entered patients); RO resections were performed in 60% of the cases, mainly after an objective response (13/15 versus 4/12 in nonresponders). No pathological CR were seen. Grade 4 neutropenia was observed in eight cycles (5 patients), with five septic complications and one death due to toxicity. Four postoperative complications were observed: 2 cases of severe pneumonia and 2 subphrenic abscesses. One postoperative death, due to intravascular disseminated coagulation, was observed at day 30. Median survival was 16 months and the 1-, 2- and 3-year survival was 67, 42 and 38%, respectively. Patients with linitis plastica had a significantly shorter survival (P < 0.002). We conclude that neodjuvant chemotherapy is feasible in LAGC, although randomised trials are warranted to demonstrate its efficacy on survival and resection rates.
The most common type of gastric metastases secondary to carcinoma of the breast is the one showing a linitis plastica appearance. The roentgenologic aspect when performing an upper gastrointestinal examination with barium contrast is typical and allows the differential diagnosis with the primitive linitis. The linitis plastica type of metastatic breast carcinoma results from transmural infiltration of the stomach wall with initial deposit of tumor cells in the submucosal or subserosal regions. CT provide valuable information concerning the metastatic spread.
We describe the computed tomography (CT) appearances of three cases of antroduodenal linitis plastica metastases from breast carcinoma. Two of the three cases had biliary obstruction as a consequence and required endoscopic stenting. Antroduodenal linitis plastica should be considered as a possible cause for jaundice in patients with breast carcinoma.
A 55-year old man with von Recklinghausen's disease was admitted to our hospital for investigation of weight loss, anorexia, and abdominal discomfort. Endoscopy findings revealed impairment in the extendability of the stomach by air, and barium meal studies showed a well-contracted stomach. On exploratory laparotomy, the whole of the stomach was found to be grossly shrunken with thickening of the walls suggesting a "linitis plastica" stomach. Histological findings confirmed stage III poorly differentiated diffuse infiltrative adenocarcinoma of the stomach. This report describes a rare case of "linitis plastica" stomach in a patient with peripheral neurofibromatosis (NF-1) indicating that the NF-1 disorder does not spare an individual from other common neoplastic disorders.
Gastric carcinoma of the linitis plastica type is occasionally difficult to diagnose endoscopically because of the large inflammatory response and the sparsity of tumor cells. Five patients who presented with signs and symptoms of gastric carcinoma underwent upper gastrointestinal endoscopy to confirm the diagnosis of carcinoma. In each case the gross appearance of the stomach was felt to represent gastric carcinoma but the biopsy and/or brushing specimens were unable to make the diagnosis. Ultrasound or CT in each of these five patients demonstrated thickening of the gastric wall and, in one instance, evidence of extensive metastatic disease. Fine needle aspiration biopsy was performed and a diagnosis of adenocarcinoma was made cytologically. Four were primary gastric adenocarcinoma of the linitis plastica type and one was metastatic adenocarcinoma from the breast. It is suggested that guided aspiration biopsy be performed when the diagnosis of gastric carcinoma cannot be confirmed endoscopically.
PURPOSE: The purpose of this work was to describe the helical CT findings of intestinal metastasis from gastric adenocarcinoma. METHOD: Twenty-three patients with intestinal metastasis from gastric adenocarcinoma found at helical CT were included. CT findings and clinical and pathologic data were reviewed. RESULTS: The most common characteristic finding was target-like concentric bowel wall thickening (thick inner high-outer low, n = 18) involving multiple long segments with progressive thickening of the enhancing inner layer. Fifteen cases (65%) involved multiple sites, and the ascending colon (n = 12) and rectum (n = 11) were the two most common sites. Peritoneal carcinomatosis (n = 15, 65%) and bowel obstruction (n = 14, 61%) were common associated findings. Regarding the primary lesion, the majority was linitis plastica (n = 16) and poorly differentiated adenocarcinoma with or without signet-ring cell differentiation (n = 14). CONCLUSION: Intestinal metastasis from gastric adenocarcinoma, especially of the linitis plastica type, most commonly showed target-like long segmental wall thickening with a characteristically thick inner enhancing layer on helical CT.
Gastric adenocarcinomas, even in the absence of distant metastases, have a poor prognosis which is particularly dismal when tumors are located in the cardia, in the event of locoregional lymph node involvement and/or bulky tumors. Postoperative adjuvant chemotherapy has never clearly demonstrated its efficacy on survival. Besides ongoing trials using new and more active regimens, preoperative chemotherapy has been used for unresectable cancer due to loco-regional extension and when locally advanced cancer is potentially resectable but with poor prognosis such as bulkiness, when tumors are located in the cardia and when there is tumor in the coeliac area at CAT-scan with suspected metastatic lymph nodes. In case of unresectable tumor at initial surgery five publications have reported the ability of chemotherapy to reduce the tumor volume and to allow subsequent resection of the gastric tumor in 40% to 60% of the cases. In these cases there is a clear survival advantage as the median survival reported in 2 of these studies was 12 and 18 months compared to the 4 to 6 months median survival reported in historical studies in case of unresectable cancer [17, 18]. In case of locally advanced gastric tumors some Japanese case reports have demonstrated the ability of preoperative chemotherapy to concentrate in the tumor tissue and to downstage the tumors. Four North American and European studies have demonstrated that preoperative chemotherapy is feasible, and will probably increase the resection rate. J. Ajani has reported 2 studies in which tolerance was acceptable: a major response (R) observed in 24% and 31%, the resectability rates were 72% and 77% and the median survival 15 and 16 months, respectively. Our experience is based on 30 patients treated with a combination of continuous i.v. 5-FU and CDDP. Fifteen had a tumor of the cardia, 15/30 had enlarged lymph nodes and 7/30 a linitis plastica (diffuse type). After a mean number of 3 cycles, 27/30 patients were evaluable for response. One patient achieved a CR and 14 a PR (OR rate 56%, 95% CI: 38% to 74%) but only one of those with linitis plastica responded. Twenty-eight patients underwent surgery and 23 had a macroscopically complete resection (82%). Resectability rate was higher after OR (13/15) than in nonresponding patients (4/12). Toxicity was acceptable, however grade 4 leucopenia in 5 patients and one toxicity-related death were observed. There was no increase in postoperative complications. Nine patients received postoperative chemotherapy and 3 patients with positive margins received postoperative external radiotherapy.(ABSTRACT TRUNCATED AT 400 WORDS)