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At least 649 records · Page 36Linked to original sources
PubMed1998

Colorectal and extracolonic cancer variations in MLH1/MSH2 hereditary nonpolyposis colorectal cancer kindreds and the general population.

PURPOSE: This clinical case review aimed to identify phenotypic variations in colorectal and extracolonic cancer expression between hereditary nonpolyposis colorectal cancer (HNPCC) families with MLH1 and MSH2 germline mutations and the general population. METHODS: Colorectal cancer onset and site distribution were compared among 67 members of MLH1 kindreds, 45 members of MSH2 kindreds, and 1,189 patients from the general population. Synchronous and metachronous cancer rates, tumor stage, extracolonic cancer incidence, and survival were also compared. RESULTS: Mean ages of colorectal cancer onset were 44, 46, and 69 years for MLH1, MSH2, and the general population, respectively (P < 0.001). More proximal and fewer distal colon cancers were noted in HNPCC than the general population (P < 0.001, P = 0.04). Site distribution showed disparity of rectal cancers (8 percent MLH1 vs. 28 percent MSH2; P = 0.01) based on genotypes. Overall, synchronous colorectal cancer rates were 7.4, 6.7, and 2.4 percent for MLH1, MSH2, and the general population, respectively (P = 0.016). Annual metachronous colorectal cancer rates were 2.1, 1.7, and 0.33 percent for MLH1, MSH2, and the general population, respectively (P = 0.041). Colorectal cancer stage presentation was lower in HNPCC than the general population (P = 0.0028). Extracolonic cancers were noted in 33 percent of MSH2 patients, compared with 12 percent of MLH1 patients and 7.3 percent of the general population with colorectal cancers (P < 0.001). Combined MLH1 and MSH2 ten-year survival was 68.7 percent compared with 47.8 percent for the general population (P = 0.009 stage stratified, hazard ratio 0.57). CONCLUSION: The presence of rectal cancer should not preclude the diagnosis of HNPCC, because the incidence of rectal cancer in MSH2 was comparable with that in the general population. Phenotypic variations, including the preponderance of extracolonic cancers in MSH2 patients, did not result in survival differences between genotypic subgroups. These phenotypic features of HNPCC genotypes may have clinical significance in the design of specific screening, surveillance, and follow-up for affected individuals.

Adaptor Proteins, Signal Transducing↗
PubMed1985

Radiation-induced esophageal carcinoma responded well to hyperthermic chemotherapy--a case report.

A 66-year-old man with multiple malignant lesions of the esophagus, as apparently induced by irradiation of 60 Gy of 60Co for a mediastinal tumor at the age of 36 y.o., was a poor operative risk because of severe lung disease. This man was treated with a combination of hyperthermia, oral administration of oil Bleomycin-polyacrylate paste and intravenous infusion of cis-Platinum. During 20 administrations of hyperthermia conducted at 42-45 degrees C for 30 min, 600 mg of oil Bleomycin and 200 mg of cis-Platinum, no complications occurred and the heat treatments were completed with no side effects. Repeatedly performed esophagogram and endoscopy showed a complete disappearance of three of the four lesions and a marked regression in the other one. There has been no regrowth or distant spread of the carcinoma up to the present 10 months of follow up. This combination of hyperthermia and chemotherapy may prove to be the optimal strategy for treatment of unresectable esophageal cancer, particularly when radiotherapy is contraindicated.

Aged↗
PubMed1999

Comparison of p53 and bcl-2 expression in initial, synchronous, and metachronous colorectal adenomas.

To investigate the significance of p53 and bcl-2 expression in metachronous colorectal adenomas arising in the remaining colon after carcinoma resection, we analyzed p53 and bcl-2 expression immunohistochemically in initial adenomas (type I), synchronous adenomas with concurrent carcinoma (type II), and metachronous adenomas arising after resection of initial adenomas (type III) or carcinoma (type IV). The incidence of p53 immunoreactivity in type IV adenomas with mild dysplasia was significantly higher than that in type I, type II, or type III adenomas with mild dysplasia. bcl-2 immunoreactivity was more frequently detected in type IV adenomas with mild dysplasia than in type I or type III adenomas with mild dysplasia. Coexpression of p53 and bcl-2 was detected in 16% of the type IV adenomas, this being a significantly higher frequency than that seen in the type I, type II, or type III adenomas. These results suggest that the evaluation of p53 and bcl-2 in metachronous adenomas in the remaining colon after resection of carcinoma may be a useful biologic marker for assessing the risk of cancer development.

Adenoma↗
PubMed1998

Leiomyoma of the urinary bladder.

Leiomyoma of the urinary bladder is a rarity but should be considered in the differential diagnosis of intramural neoplasm. We report a case illustrating clinical and pathological features in particular the immunohistochemistry. Etiology and differential diagnosis are discussed.

Diagnosis, Differential↗
PubMed2001

Myelodysplastic syndrome progresses rapidly into erythroleukemia associated with synchronous double cancers of the stomach and the papilla of Vater.

Patients with myelodysplastic syndrome (MDS) show a relatively high incidence of developing cancers. However, it is extremely rare that synchronous double cancers develop in an MDS patient. We report a case of MDS that progressed rapidly into erythroleukemia (M6 by French-American-British classification) complicated by gastric cancer and carcinoma of the papilla of Vater. A 66-year-old man was admitted because of pancytopenia with peripheral blasts. A diagnosis of MDS (with refractory anemia with excess of blasts in transformation [RAEB-T]) was made by bone marrow examination. Chromosome analysis revealed 46,XY. An early gastric cancer was also diagnosed by endoscopic examination. The peripheral blasts gradually proliferated and the disease progressed to M6. A chromosome abnormality 46,XY,del(1)(q42) was detected at the leukemic transformation. A CAG (low-dose cytarabine and aclarubicin in combination with granulocyte colony-stimulating factor) regimen was started as a remission-induction therapy. However, obstructive jaundice developed and a marked dilatation of bile ducts was observed by abdominal computed tomography (CT). A carcinoma of the papilla of Vater was detected by endoscopy. As remission was achieved and the pancytopenia improved, the patient subsequently underwent a surgical jejuno-choledochostomy to manage the jaundice. However, the leukemia relapsed thereafter and additional chromosome abnormalities including der(5)t(5;10)(p15:q11) were observed.

Aclarubicin↗
PubMed2003

[Progressive paralysis caused by radiation-induced cervical malignant peripheral nerve sheath tumor].

HISTORY AND DIAGNOSIS: A 59-year old engineer was admitted to the hospital because of pain in his right collar region and the onset of incomplete paresis of the right arm. Magnetic resonance tomography displayed an advanced tumour arising from the right paravertebral soft tissue. Histological examination revealed a malignant peripheral nerve sheath tumor (MPNST). Thirteen years before admission, the patient had a right-sided tumor-tonsillectomy of a squamous cell carcinoma and local radiation of a cystic squamous cell carcinoma in the ipsilateral cervical soft tissue. CLINICAL COURSE AND THERAPY: In the following course, progressive neurological symptoms occurred including beginning paraplegia, right phrenic paralysis and a severe concomitant pain syndrome. Due to the location and advanced tumor state, surgical treatment was not performed and palliative chemotherapy remained ineffective. Three months later the patient died due to rapidly progressive neurological failure. CONCLUSION: MPNST represents a rare entity which has been related to postoperative radiation. Unusual neurological symptoms in anatomical regions of former radiation should therefore include neurogenic secondary malignancies in the differential diagnosis for early surgical intervention.

Arm↗
PubMed2004

Bone marrow transplantation for therapy-related acute myeloid leukemia in congenital retinoblastoma associated with 13q deletion syndrome.

Children with constitutional deletion of the long arm of chromosome 13 are at risk for retinoblastoma (RB) due to loss of the RB tumor suppressor gene. The prognosis is poor since the tumors are often bilateral, aggressive, and recurrent and the patients often harbor other congenital abnormalities. One further complication is that of therapy-related malignancies later in life. We report a case of allogeneic stem cell transplantation for therapy-related acute myeloid leukemia in an 8-year-old girl after multimodality treatment for refractory bilateral relapsing RB, with excellent outcome in both the ophthalmic and marrow disease.

Acute Disease↗
PubMed2006

Laparoscopic versus open resection for appendix carcinoid.

BACKGROUND: Since an increasing number of appendectomies are performed via laparoscopy, it is crucial to determine the impact of this approach on appendix carcinoid (AC) outcome. The goal of this study was to compare results of laparoscopic (LAP) versus open (OP) appendectomy for AC according to intend to treat approach. METHODS: A retrospective review (1991-2003) identified 39 patients (median age, 36 years; range, 12-83) treated by laparoscopy (LAP) or laparotomy (OP) for AC in a single institution. Follow-up was complete for all patients (median, 67 months; range, 4-132). RESULTS: Most cases had associated acute appendicitis (64%). Median carcinoid size was 1.1 cm (range, 0.3-5) and 0.4 cm (range, 0.2-3) in the LAP and OP groups, respectively. LAP and OP were performed in 21 (54%) and 18 (46%) patients, respectively. Surgical margins were positive in two patients in the LAP group and one patient in the OP group (p = 0.6). Right colectomies were performed for AC >2 cm in five patients after LAP and in four patients after OP (p = 0.9). Actuarial 5-year survival rates were 100 and 94% in the LAP and OP groups, respectively (p = 0.2). Two patients died in the OP group, one due to metastatic carcinoid and the other due to metachronous colorectal cancer. Synchronous or metachronous colorectal carcinomas developed in six patients (15%). CONCLUSION: Laparoscopic appendectomy is a safe procedure for AC, with carcinologic and long-term results similar to those of conventional appendectomy. Thus, pre- or per-operative suspicion of AC is not a contraindication to LAP. Prognosis of AC appears more dependent on carcinoid malignant potential or associated tumors. Risk for developing colorectal adenocarcinoma is high in AC patients and warrants follow-up of all patients with colonoscopic screening.

Adolescent↗
PubMed2000

Synchronous or metachronous double cancers of the pancreas and other organs: report on 12 cases.

Pancreatic carcinoma carries a poor prognosis, especially invasive ductal carcinoma of the pancreas. This retrospective study describes the results of the treatment and prognosis for double cancers in which cancer of the pancreas was associated with malignancies in other organs in 12 patients who were diagnosed and treated at Kurume University Hospital. The patients included 4 women and 8 men, with an average age of 67 years. Of the 12 tumors, 7 were metachronous pancreatic cancers which occurred after resections of other organ malignancies. Five patients had synchronous double cancers, one of whom was diagnosed to have gastric cancer on admission. Two other patients of this group were diagnosed to have lung cancer, while the remaining 2 patients suffered from colon cancer. By the time pancreatic cancer was diagnosed, gastrectomies had been performed in 7 patients for either gastric cancer or ulcers. In addition, one patient underwent a hysterectomy for uterine carcinoma and another received a low anterior resection for rectal carcinoma. Four of 5 patients in the synchronous group had nonresectable tumors and a palliative bypass operation was performed in 2 of these patients. Six patients who had metachronous double cancers died because of pancreatic cancer recurrence or metastases. We conclude that the prognosis of double cancers, where cancer of the pancreas is associated with other organ malignancies, primarily depends on the prognosis of the pancreatic carcinoma, and the present study suggests the necessity of long-term follow-up examinations for gastrectomy patients in order to make an early diagnosis of pancreatic cancer.

Aged↗
PubMed2003

Patterns of surgery in patients belonging to amsterdam-positive families.

INTRODUCTION: The phenotype of hereditary nonpolyposis colorectal cancer includes an 80 percent lifetime risk of colorectal cancer, a predominance of lesions proximal to the splenic flexure, and a high incidence of synchronous and metachronous neoplasia. Although prophylactic colectomy is rarely advised for patients with a hereditary nonpolyposis colorectal cancer genotype and a normal colon, the presence of advanced neoplasia in the context of a qualifying family history or a hereditary nonpolyposis colorectal cancer genotype has led to such recommendations. We performed this study to document the patterns of colorectal surgery performed for cancer-bearing patients who are part of an Amsterdam criteria-positive family and to compare rates of metachronous cancers that follow index total or segmental colectomy. METHODS: Family trees fulfilling the classic Amsterdam criteria for hereditary nonpolyposis colorectal cancer were identified, and all patients for whom surgical and pathology records were available were included in the study. Type of surgery and the outcome of subsequent follow-up were abstracted. Patients were divided into those treated at the Cleveland Clinic and those treated elsewhere. RESULTS: There were 39 families with 93 affected patients. These patients had 127 colorectal cancers, 76 (60 percent) of which were right sided (proximal to the splenic flexure). Median age at diagnosis of the index cancer was 47 (range, 26-81) years. Sixteen patients (17 percent) had metachronous cancers and multiple surgeries, whereas four (4 percent) had synchronous cancers. Median follow-up for patients who underwent surgery at the Cleveland Clinic was 13 (range, 4-49) years, whereas that for those who underwent surgery elsewhere was 14 (range, 1-42) years. Sixteen (48 percent) of the 33 patients who underwent surgery at the Cleveland Clinic had a total colectomy vs. 7 (12 percent) of the 60 who had surgery elsewhere (Fisher's exact test, P < 0.001). Only one patient who had surgery at the Cleveland Clinic had a second operation for a metachronous cancer (1/17 patients having a segmental resection). Fifteen patients who underwent surgery elsewhere needed a second resection for metachronous cancer (15/53 patients having a segmental resection; Fisher's exact test, P = 0.094). CONCLUSION: We conclude that there is high risk of metachronous colorectal cancer if an index cancer in a hereditary nonpolyposis colorectal cancer patient (defined according to Amsterdam criteria) is treated by partial colectomy. However, this risk can be lowered by performing a total colectomy at the time of index surgery, or possibly by effective postoperative surveillance.

Adult↗
PubMedDate not supplied

Feasibility of breast conservation therapy in metachronous or synchronous bilateral breast cancer.

BACKGROUND: The optimal management of contralateral breast cancer (CC) in patients previously treated with breast-conserving therapy (BCT) is unclear, as is whether these patients continue to choose BCT as the preferred treatment of their second breast cancer. METHODS: Of 1328 patients treated with BCT at The University of Texas M. D. Anderson Cancer Center between 1958 and 1994, 63 developed a contralateral breast cancer. We reviewed the charts of these patients retrospectively, and standard demographic and treatment variables were evaluated. Survival was analyzed by the Kaplan-Meier method and subgroups by chi2 analysis. RESULTS: Twenty-nine percent of the patients had a family history of breast cancer. First breast cancers were detected by patient or physician in 67% of cases and by mammogram in 17% of cases, compared to 59% and 36%, respectively, of CC (P = .04). Median time to development of CC was 61 months. Sixty percent of the initial tumors were AJCC stage 0 or I with a median size of 2 cm, whereas 74% of the CC were stage 0 or I (P = .02), with a median size of 1.5 cm. Eighty-seven percent of patients chose BCT for treatment of CC. There were few treatment-related complications. Recurrence rates were not significantly different from those of patients undergoing BCT for the initial cancer (P = .47), and 5- and 10-year actuarial survival rates after the first cancer were 93% and 76%, respectively. Median follow-up was 134 and 56 months from the time of diagnosis of the initial cancer and CC, respectively. CONCLUSIONS: Because contralateral breast cancer often is detected at an early stage, there are few treatment-related complications, and the risk of recurrence is no different from that for the initial cancer, BCT is an acceptable and desirable option for appropriately selected patients with metachronous or synchronous bilateral breast cancers.

Adult↗
PubMed2005

Gliomatosis cerebri and pituitary adenoma: case report and literature review.

The diagnosis of Gliomatosis cerebri (GC) is known to be difficult and is still a matter of debate. We describe an in vivo case of GC associated with a pituitary tumor. A 47-year-old woman presented with short-term memory loss. A MRI revealed the presence of a pituitary enhancing tumor and a diffuse lesion involving the brain. A left pterional craniotomy with partial temporal lobectomy and removal of the pituitary lesion were performed in order to obtain diagnosis. The histological analyses showed a pituitary non-functioning tumor and a GC consisting of neoplastic oligodendrocytes and astrocytes. Both lesions showed nuclear immunoreactivity for progesterone receptors (PGr) and estrogen receptors (EGr). This result could suggest there is a common receptor substrate in these tumors. In this case hormones could constitute a common step in tumorigenesis of both lesions.

Adenoma↗
PubMed1980

Neural tumors of the oral cavity. A review of the spectrum of benign and malignant oral tumors of the oral cavity and jaws.

The clinical and histologic features of benign and malignant neural tumors of the oral cavity and jaws are reviewed. Some rarer histologic variants are mentioned. Particular attention is paid to the two syndromes involving neural tumors of the oral cavity, namely, neurofibromatosis and multiple endocrine neoplasia III. A previously unreported case of the latter is presented.

Amputation Stumps↗