Maria Isete Fares Franco

Heterotopia pancreática: análise clínico-patológica de 18 doentes

OBJECTIVE To analyze the clinical and pathological features of heterotopic pancreatic tissue in abdominal digestive organs. METHODS We retrospectively studied 18 patients with histologically diagnosed heterotopic pancreas. Clinical and histopathologic data were reviewed. Heterotopic pancreatic tissues were classified in three histological models: Type I consists of three components of normal pancreas (acini, ducts and islets), type II with two components and type three with only one component. RESULTS The mean age was 52.7 years, ranging from 34 to 73 years, nine of them men and nine women. Symptoms were observed in only four patients, and their lesions were diagnosed by gastroscopy. The remaining 14 were asymptomatic and their anomalies were discovered accidentally. Most of the lesions were located in the upper gastrointestinal tract: seven (38.9%) in the stomach, six (33.3%) in the duodenum and three (16.6%) in the jejunum. Heterotopia was mostly located in the submucosa (83.3%) but was also observed in the muscularis propria and in the sub-serosa. In seven specimens (38.9%) all pancreatic components were found (type I), in eight (44.4%) exocrine glands and excretory ducts were present (type II) and in three (16.7%) only exocrine tissue was observed (type III). CONCLUSION Pancreatic heterotopia is rare. Patients with pancreatic ectopia diagnosed by pathological study, whether asymptomatic or with mild symptoms, should be observed. Lesions incidentally detected during surgeries need to be removed by conservative procedures.

CARCINOID TUMOR OF THE DUODENUM: a rare tumor at an unusual site. Case series from a single institution

CONTEXT Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated. OBJECTIVE To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids. METHODS Twenty patients (12 females and 8 males) were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old). The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011) were analyzed. RESULTS The most common symptoms were dyspepsia (50%) and epigastric pain (45%) followed by weight loss (10%) and vomiting (5%). Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75%) patients, the second part in 4 (20%) patients, and the third part in 1 (5%) patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95%) patients and an histopathological examination of the surgical specimen in 1 (5%) patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm). Nineteen (95%) patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%), whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20%) patients. Four (20%) patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80%) cases, and it penetrated into the muscular layer in 4 (20%) cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months). Twelve (60%) of the 20 cases in this series are alive without any evidence of active disease. Only one (5%) patient died due to liver metastases of the duodenal carcinoid. CONCLUSIONS Duodenal carcinoids are rare and indolent tumors usually associated with a benign progression. Duodenoscopy, computerized tomography, and endoscopic ultrasound should be performed to evaluate the tumor size, the level of wall invasion, and the presence of regional or distant lymphatic metastases. Endoscopic removal of tumors smaller than 1.0 cm without periampullary localization or evidence of muscular propria layer invasion assessed by histology and/or endoscopic ultrasound is recommended. The endoscopic resection with a carcinoid tumor size between 1.0 cm and 2.0 cm can be incomplete and require new endoscopic resection or even surgical removal. Duodenal carcinoid larger than 2.0 cm require full-thickness resection and concomitant lymphadenectomy.

Intestinal metaplasia in gallbladders: prevalence study

CONTEXT AND OBJECTIVE Gallbladder cancer is usually diagnosed at a late stage and generally results in death. Discovery of predisposing factors for this neoplasia could prevent this outcome. In this study, we assess the presence of one of these factors: intestinal metaplasia in gallbladders with stones and inflammatory processes. DESIGN AND SETTING Cross-sectional study in Hospital do Servidor Público Estadual de São Paulo. METHOD The first 80 gallbladders from patients who underwent elective cholecystectomy between April and August 2002, presenting stones and chronic inflammation, were studied. The patients were divided into groups according to their age: CC1, from 15 to 40 years; CC2, from 41 to 60 years; and CC3, from 61 to 85 years. RESULTS Twenty-one patients (26%) were male, while 59 (74%) were female. In the group CC1, intestinal metaplasia was present in 85.71% of the 21 patients studied; in CC2, in 79.41% of 34 patients; and in CC3, in 56.00% of 25 patients. These differences presented statistical significance (p = 0.04542). CONCLUSION Intestinal metaplasia is extremely frequent in gallbladders with inflammation and lithiasis, especially in younger patients.

Colonic carcinoid tumors: a clinicopathologic study of 23 patients from a single institution

CONTEXT Colonic carcinoids, excluding those arising in the appendix, have proved to be extremely rare. Due to their rarity, the characteristics and behavior of this unusual malignancy remain unclear. OBJECTIVE To review the clinicopathologic features of patients operated on carcinoid tumors of the colon. METHODS Twenty-three patients (12 males and 11 females) were operated on colonic carcinoids. The mean age of the patients was 63.0 +/- 12.9 years (42 to 85 years). The clinical and histopathological data of patients who were pathologically diagnosed as having carcinoid tumors and submitted to surgical treatment over a 30-year period (1977-2007) were gathered. Actuarial patient survival was estimated using the Kaplan-Meier method, with carcinoid-specific death as the outcome. RESULTS The mean time elapsed between onset of symptoms and surgical treatment was 8.3 months (1.5 to 20 months). The most frequent symptoms or signs encountered were abdominal pain followed by anorexia or weight loss, diarrhea, abdominal tenderness, palpable abdominal mass, and rectal bleeding. No carcinoid syndrome was noted. The lesion was located in the cecum in 16 (69.6%) patients, in the sigmoid in 3 patients (13.0%), in the ascending colon in 3 patients (13.0%), and in the transverse colon in one patient (4.3%). Twenty-one (91.3%) patients were operated on curative intent. Spreading of the disease to the liver and peritoneum was found in two (8.7%) patients who submitted to intestinal bypass. The mean size of the largest mass was 3.7 +/- 1.2 cm (1.5 to 6.2 cm). There were multiple (two or more) lesions in three cases (13.0%). In the resected cases, the lymph nodes were compromised in 10 patients (47.6%) and disease-free in 11 (52.4%). Venous invasion and neural infiltration were both present in five (23.8%) patients. The tumors had penetrated the muscularis propria in all resected cases. Four (17.4%) patients had a second non-carcinoid primary tumor. Three (13.0%) patients died due to postoperative complications and five (21.7%) patients died from metachronous metastases or local recurrence. Fifteen patients (65.2%) remain alive without evidence of active disease. The mean follow-up period was 12 years (1.2 to 18 years), whereas the mean global survival was 50.7 +/- 34.2 months and the crude survival rate at 5 years was 62.7%. CONCLUSIONS Carcinoid tumors of the colon are frequently right-sided and may be clinically occult until an advanced stage is reached. Based on the relatively poor survival rates reported, it is recommended that, in addition to standard surgical resection, vigorous surveillance for metastatic disease must be performed, particularly during the first 2 years after surgery. In addition, these patients require evaluation of the entire gastrointestinal tract for evidence of coexisting malignancy, along with an extended period of follow-up, because tumor recurrences after 5 years are not uncommon.

Multiple lymphomatous polyposis of the gastrointestinal tract

CONTEXT Gastrointestinal multiple lymphomatous polyposis is a rare type of malignant lymphoma that has aggressive biological behavior, early systemic dissemination and poor prognosis. It is considered to be a manifestation of non-Hodgkin lymphoma and represents the gastrointestinal counterpart of mantle cell nodal lymphoma. OBJECTIVE A case of gastrointestinal multiple lymphomatous polyposis is presented and the anatomopathological, clinical, diagnostic and treatment aspects of this unusual neoplasia are discussed. CASE REPORT The patient was a 59-year-old white male with a complaint of asthenia, night sweating, alteration in intestinal habit and weight loss over the preceding two months. The physical examination showed pallid mucosa and a palpable mass in the epigastrium and mesogastrium. Endoscopy of the upper digestive tract showed the presence of gastric and duodenal polyps. An opaque enema showed multiple polypoid lesions, especially in the cecum. A rectal biopsy revealed infiltration of the mucosa and submucosa by diffuse lymphoma consisting of small cleaved cells. Immunohistochemical study showed lymphocytes that expressed the antibody CD20 (L-26) and light-chain kappa (k) immunoglobulin, but not light-chain lambda (l) immunoglobulin. The patient presented a condition of acute intestinal obstruction with the presence of a mesenteric mass formed by agglutinated lymph nodes that surrounded the proximal ileum, thereby obstructing its lumen. He was submitted to a segmental enterectomy and gastrotomy with excisional biopsies of the gastric polypoid lesions. After two cycles of chemotherapy there was a worsening of the general state, with an increase in the dimensions of the abdominal masses and sepsis, accompanied by progressive respiratory insufficiency, leading to death.

Retroperitoneal unicentric Castleman's disease (giant lymph node hyperplasia): case report

CONTEXT AND OBJECTIVE Castlemans disease, or giant lymph node hyperplasia, is a rare disorder of the lymphoid tissue that causes lymph node enlargement. It is considered benign in its localized form, but aggressive in the multicentric type. The definitive diagnosis is based on postoperative pathological findings. The aim here was to describe a case of retroperitoneal unicentric Castlemans disease in the retroperitoneum. CASE REPORT A 61-year old white male with weight loss and listlessness presented with moderate arterial hypertension and leukopenia. Abdominal tomography revealed a 5 x 4 x 5 cm oval mass of low attenuation, with inner calcification and intense enhancement on intravenous contrast, located in the retroperitoneal region, between the left kidney and the aorta, at the renal hilus. Exploratory laparotomy revealed a non-pulsatile solid oval mass situated in the retroperitoneum, adjacent to the left renal hilus. The retroperitoneal lesion was removed in its entirety. Examination of frozen samples revealed benign lymph node tissue and histopathological examination of the surgical sample revealed hyaline-vascular giant lymph node hyperplasia (Castlemans disease). The patient was discharged on the 12th day without significant events. Two months after the operation, the patient was readmitted with severe cardiac insufficiency, acute renal failure and bronchopneumonia, which progressed to acute respiratory insufficiency, sepsis and death.

Tratamento cirúrgico do linfoma gástrico primário

BACKGROUND: we analyzed the results from 25 patients with primary gastric lymphoma operated with curative intention. METHODS: Data were obtained retrospective ly and by contacting patients or theirs relatives. The disease was staged using the Ann Arbor system for non-Hodgkins lymphoma, as modified by Musshoff and Schmidt-Vollmer, and classified according to Kiels system. RESULTS: The signs and symptoms were similar to those of peptic ulcer disease or gastric carcinoma. Preoperative diagnosis was obtained by endoscopic biopsy in three cases and by surgical exploration in the remaining. Seven patients (28%) were submitted to myelograms, which were normal in all cases. All patients were submitted to resection (12 subtotal gastrectomies and 13 total gastrectomies) with removal of regional lymph nodes. Ten of them (40%) received complementary treatment (chemotherapy and/or radiotherapy). The staging was significantly more advanced in fundocardiac lesions and in more elderly patients, and the average survival was 31.5 months. CONCLUSIONS: in this series, the variables that influenced significantly survival rate were age, advanced stage, size of the lesion higher than 6,0 cm, and adjuvant postoperative treatment (p< 0,05). These results suggest that complete resection of the lesion with the adjacent lymph nodes, accompanied by adjuvant treatment, constitutes the best approach to resectable primary gastric lymphoma.

Extensive colorectal lymphomatous polyposis complicated by acute intestinal obstruction: a case report

BackgroundMultiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine. Multiple lymphomatous polyposis originates from the mantle zone of the lymphoma follicle and is considered to be a mantle cell lymphoma, which is a relatively aggressive type of B-cell non-Hodgkin’s lymphoma. We report an unusual case of a patient with multiple lymphomatous polyposis with extensive colorectal involvement and acute intestinal obstruction, an atypical complication of this rare disease. On the basis of this case study, the pitfalls in gastrointestinal tract lymphomatous polyposis diagnosis and prognosis, as well as the treatment options, are discussed.Case presentationOur patient was a 76-year-old white woman with asthenia, cramps, and swelling in the lower left quadrant of the abdomen, as well as weight loss within the previous 5 months. A colonoscopy revealed polyps in the rectum, sigmoid colon, descending colon, and right and left colic flexures. A biopsy revealed lymphomatous infiltration of the intestinal wall. Because of the large size of the polypoid masses, which narrowed the colonic lumen in multiple locations, the patient developed acute intestinal obstruction and was referred for laparotomy. She underwent a total proctocolectomy with a permanent ileostomy and a left salpingo-oophorectomy. Microscopic examination showed the presence of a multicentric, low-grade, small lymphocytic lymphoma. Immunohistochemical analysis revealed positive immunostaining for CD79a, CD20, and CD45. These results were consistent with the diagnosis of mantle cell lymphoma. Two weeks after surgery and prior to discharge, but before the beginning of chemotherapy, the patient’s general condition worsened as she experienced a severe and progressive respiratory tract infection, advanced respiratory insufficiency, and septic shock, and she ultimately died.ConclusionsMantle cell lymphoma develops as a progressive and aggressive disease with widespread polyposis of the gastrointestinal tract. The intensive chemotherapeutic regimens usually result in the regression of macroscopic and microscopic lesions; however, remissions are short in duration, and the median length of patient survival is 3–4 years. Mantle cell lymphoma is a rare disease that should be part of the differential diagnosis of polypoid diseases of the large intestine.

Metástase esplênica isolada de adenocarcinoma do sigmoide: relato de caso

Solitary metastatic metastasis from colorectal neoplasms is rare. Only 41 cases have been reported in the English literature until 2007. Most patients are asymptomatic, and the diagnosis is usually done by imaging studies or CEA (carcinoembrionic antigen) blood increases, which are required in the postoperative follow-up period. Case report: A 54-year-old man underwent an extended left colectomy for sigmoid colon cancer. The tumor was staged as T3N0M0. During ten months of the follow-up period, the patient remained asymptomatic with normal levels of laboratory tests, including CEA measurement. Then, there was a significant elevation of CEA, and the abdomen computed tomography revealed a mass in the spleen considered as an isolated metastasis. The patient underwent splenectomy. Histological diagnosis confirmed a metastatic adenocarcinoma with no lymph nodes invasion. The patient has been symptom-free during the 14 months of follow-up with normal blood CEA levels and negative radiological studies. CONCLUSION: Solitary spleen metastasis from colon carcinoma is rare, and splenectomy provides an expressive improvement in the survival.