A síndrome de Budd-Chiari não tem sido descrita em pacientes jovens portadores de câncer gástrico. Relata-se caso de síndrome de Budd-Chiari e linfangite carcinomatosa em homem branco de 28 anos, portador de câncer gástrico insuspeito, apresentando icterícia, hematêmese e dispnéia. O estudo de autopsia revelou adenocarcinoma gástrico infiltrando vasos das camadas submucosa e serosa, com sangramento do estômago e intestino, além de metástases hepáticas e pulmonares. Múltiplos microtrombos mistos (fibrina, plaquetas e células tumorais) foram observados em pequenos vasos sangüíneos pulmonares e tanto os vasos linfáticos subpleurais quanto o interstício pulmonar continham células tumorais metastáticas.The Budd-Chiari syndrome has not been described in young patients with gastric cancer. A case of Budd-Chiari syndrome and carcinomatous lymphangitis is reported in a 28 years-old white man with unsuspected gastric cancer, presenting jaundice, hematemesis and dyspnea. Autopsy disclosed gastric adenocarcinoma invading vessels of the submucous and serous layers, with gastric and intestinal bleeding, liver and lung metastases. Multiple mixed (fibrin, platelets and tumor cells) microthrombi were observed in small pulmonary blood vessels, and both subpleural lymph vessels and lung interstitium contained metastatic tumor cells

Araújo, Ana C.

Castro, Eumênia C. C.

Reis, Marlene A.

Santos, Vitorino M.

Teixeira, Vicente P. A.

English

The Budd-Chiari syndrome has not been described in young patients with gastric cancer. A case of Budd-Chiari syndrome and carcinomatous lymphangitis is reported in a 28 years-old white man with unsuspected gastric cancer, presenting jaundice, hematemesis and dyspnea. Autopsy disclosed gastric adenocarcinoma invading vessels of the submucous and serous layers, with gastric and intestinal bleeding, liver and lung metastases. Multiple mixed (fibrin, platelets and tumor cells) microthrombi were observed in small pulmonary blood vessels, and both subpleural lymph vessels and lung interstitium contained metastatic tumor cells.A síndrome de Budd-Chiari não tem sido descrita em pacientes jovens portadores de câncer gástrico. Relata-se caso de síndrome de Budd-Chiari e linfangite carcinomatosa em homem branco de 28 anos, portador de câncer gástrico insuspeito, apresentando icterícia, hematêmese e dispnéia. O estudo de autopsia revelou adenocarcinoma gástrico infiltrando vasos das camadas submucosa e serosa, com sangramento do estômago e intestino, além de metástases hepáticas e pulmonares. Múltiplos microtrombos mistos (fibrina, plaquetas e células tumorais) foram observados em pequenos vasos sangüíneos pulmonares e tanto os vasos linfáticos subpleurais quanto o interstício pulmonar continham células tumorais metastáticas

Cadernos Espinosanos (E-Journal)

BUDD-CHIARI  SYNDROME  AND  UNSUSPECTEDGASTRIC  ADENOCARCINOMA*SÍNDROME  DE  BUDD-CHIARI  E  ADENOCARCINOMA  GÁSTRICO  INSUSPEITOVitorino M. Santos1, Eumênia C. C. Castro2, Ana C. Araújo2 , Marlene A. Reis2, Vicente P. A. Teixeira21Work from Department of Internal Medicine1 and 2Pathology Division. Triângulo Mineiro Medical School. Uberaba, MG, Brazil.Correspondence to: Profa. Dra. Eumênia Costa da Cunha Castro. Pathology Division. Triângulo Mineiro Medical School.Rua Frei Paulino, 30 Bairro Abadia. CEP 38025-180 - Uberaba-MG, Brazil.Phone - Fax: 0055 34 3126640 - E-mail: mareis.pat@dcb.fmtm.brSantos VM, Castro ECC, Araújo AC, Reis MA, Teixeira VPA. Budd-Chiari Syndrome and unsuspected gastricadenocarcinoma. Medicina (Ribeirão Preto) 2005; 38(2): 156-160.Abstract: The Budd-Chiari syndrome has not been described in young patients with gastriccancer. A case of Budd-Chiari syndrome and carcinomatous lymphangitis is reported in a 28years-old white man with unsuspected gastric cancer, presenting jaundice, hematemesis anddyspnea. Autopsy disclosed gastric adenocarcinoma invading vessels of the submucous andserous layers, with gastric and intestinal bleeding, liver and lung metastases. Multiple mixed(fibrin, platelets and tumor cells) microthrombi were observed in small pulmonary blood vessels,and both subpleural lymph vessels and lung interstitium contained metastatic tumor cells.Keywords: Budd-Chiari Syndrome. Carcinomatous  Lymphangitis. Gastric Adenocarcino-ma.  Stauffer’s Syndrome. Hepatic Vein Thrombosis.156Medicina, Ribeirão Preto,38: 156-160, abr./jun. 2005 CASO CLÍNICO1- INTRODUCTIONPatients with advanced gastric cancer maydevelop haemostatic abnormalities, as hemorrhage,thrombosis, subdural hematoma, disseminated intra-vascular coagulation (DIC) and microangiopathichemolytic anemia1/4. These changes have been relatedto paraneoplastic phenomena or bone marrow me-tastases, and may be the earliest manifestations, pre-ceding the diagnosis of cancer 2, 4.The Budd-Chiari’s syndrome (BCS), charac-terized by abdominal pain, hepatomegaly, hepatic dys-function and ascitis, has been associated with abdomi-nal cancers producing mucin and followed by severecentrilobular congestion and necrosis due to thrombo-sis or sluggish hepatic venous outflow 5, 6. BCS seemsto be less uncommon than was once believed, and mayrepresent a spectrum of disease, with varied presen-tation, depending on the balance between the rate ofthrombosis and fibrinolysis6. Since BCS has beenscarcely described in patients with gastric cancer 7,8,the finding of ascitis, hepatomegaly and abnormalitiesin liver function tests usually indicate metastaticdisease in these cases. The Stauffer’s syndrome isanother uncommon paraneoplastic condition, usuallyassociated to renal carcinoma, lymphoma or leiomyo-sarcoma9,10 that can also involve hepatomegaly,hepatic dysfunction with high liver enzymes, in*Financial support from FUNEPU157Budd-Chiari syndrome and gastric canceraddition to DIC and fibrinolysis, mimicking BCSclinical presentation. Nonetheless, the Stauffer’ssyndrome has not been described in patients with gastric adenocarcinoma.The literature revision since 1966, throughMEDLINE data base, showed that Budd-Chiari’ssyndrome has not been described in young patientswith gastric cancer. This condition has beenassociated with a high mortality rate.In the present case, severe acute liver necro-sis, due to hepatic sluggish blood flow, was the initialmanifestation of a clinically unsuspected gastricadenocarcinoma in a 28-year-old male.2- CASE REPORTA 28-year-old white male unskilled laborer cameto the hospital complaining of jaundice for a week, andabdominal pain one day before admission. He also re-ferred asthenia, dyspnea, vomiting, and a recent epi-sode of hematemesis. There was a previous history ofepisodes of  lumbar pain and hematuria, in addition toobstipation with normal colonoscopy. On admission, hewas severely ill, dyspneic, jaundiced and diaphoretic.Body mass index: 25.0kg/m2 - Temperature: 37.5oC.The heart was rhythmic, 120bpm, and there were nomurmurs. Blood pressure: 120/70mmHg. The lungswere clear. There was abdominal distension and tym-panism, with painful decompression in epigastrium. Theliver was painful and palpable 5cm below the rightcostal margin.The patient’s blood group was O, Rh-positive.The erythrocyte count 2.85x106/mm3, with polychro-masia and poikilocytosis, erythroblasts 2%, hemoglo-bin 7.9g/dl, hematocrit 26.6%, mean cell volume 93.4fl,leukocyte count 24,000/mm3 (neutrophils: myelocytes1%, bands 11%, segmented 71%, eosinophils and ba-sophils 0%, lymphocytes 14%, monocytes 3%), plate-lets 67,000/mm3, glucose 108mg/dL, urea 70mg/dL,creatinine 0.7mg/dL, sodium 138mEq/L, potassium3.9mEq/L, chloride 105mEq/L, calcium 7.9mg/dL, phos-phorous 2.7mg/dL, magnesium 1.8mg/dL, direct biliru-bin 7.25mg/dL, indirect bilirubin 1.86mg/dL, aspartatetransaminase (AST) 194U/L, alanine transaminase(ALT) 273U/L, gama glutamil transpeptidase (gGT)292U/L, alkaline phosphatase (AP) 190U/L, lacticdehydrogenase (LDH) 1,448U/L, and prothrombin ac-tivity 53%. Abdominal ultrasonography revealedhepatosplenomegaly and ascitis. Treatment includedintravenous glucose, electrolyte solutions, red blood cellstransfusion, and mechanical respiratory support. Not-withstanding, the clinical status worsened, with severedyspnea, mental confusion, progressive jaundice (bi-lirubins: direct 13.99 and indirect 3.23mg/dL) and higherliver enzymes (AST 1,351, ALT 666 and LDH 4,560U/L).On the fourth day of admission, he died due to irre-versible respiratory failure and circulatory shock.Pathologic studyAn ulcerated adenocarcinoma (diameter 2x4cm)with elevated borders and clear fundus (Fig. 1) wasfound in gastric body 7cm from pylorus. The tumorcells showed scanty cytoplasm and large polymorphicnuclei with rare mitoses, and were arranged likeglands (Fig. 2). Similar neoplastic cells were also foundin blood vessels from submucosa and serosa of thestomach. Hemorrhage through multiple punctiformlesions in the gastric mucosa, intense congestion andhemorrhagic foci in the intestinal mucosa, andhemorrhagic suffusions in the mesentery were noted.The liver weighed 2,650g, with several metastatic focishowing an aspect similar to the gastric tumor cells(Fig.3), in addition to severe diffuse congestion, necrosisand cholestatic aspect (Fig.4). The spleen weighed365g, with very intense congestion and reduced numberof lymphocytes. The lungs weighed 800g (right) and605g (left), with moderate edema in the parenchymaand hemorrhagic focuses around the trachea and inpleural surface. Multiple diffuse emboli containingtumor cells and blood lamellar material were foundin small and medium sized vessels. The gastric tumorcells were also observed in subpleural lymph vesselsand in lung interstitium. The heart weighed 347g, andthere was no evidence of arterial pulmonaryhypertension. The encephalon weighed 1,470g,showing moderate diffuse edema and hyperemia. Noneoplastic infiltration was found in the peritoneumand in the bone marrow samples.3- DISCUSSIONGastric adenocarcinoma has been rarely de-scribed in white adults under 30 years 1, and the clini-cal presentation may be very similar to many otherclinical conditions. If patient’s clinical situation wors-ens rapidly, in detriment of the differential diagnosis,the occult tumor stands to be found through autopsystudies 4, 11, 12.This 28-years-old white male with unsuspectedgastric adenocarcinoma presented severe liver dys-158Santos VM, Castro ECC, Araújo AC, Reis MA, Teixeira VPAFigure 1. Ulcerated adenocarcinoma with elevated borders andclear fundus, located in gastric body.Figure 2. Tumor cells showing scanty cytoplasm and largepolymorphic nuclei with rare mitoses, arranged like glands (arrow)(Hematoxilin-eosin x250).Figure 3. Neoplastic embolism (arrow) in central vein branches(Hematoxilin-eosin x250). Figure 4. Detail of post-sinusoidal congestion and cholestasis(Hematoxilin-eosin x200).Data from autopsy studies of thromboplastin-like producing tumors, indicate that the frequency ofthromboses is mostly age-related and associatedto disseminated bone metastases. Moreover, throm-boembolism and/or carcinomatous lymphangitis havebeen seldom described in younger patients withgastric cancer 1. In the present case, the lack of bonemarrow invasion suggests some role of paraneoplasticphenomena in the development of severe venouscongestion and acute liver necrosis. Noteworthy,gastroesophageal varices and peritoneal carcinoma-tosis were not found in this patient, which is consistentwith the diagnoses of hepatic vein thrombosis andBudd-Chiari’s syndrome.function and gastrointestinal bleeding, in addition tobreathlessness, pulmonary neoplastic thromboembo-lism and carcinomatous lymphangitis, without evidenceof bone marrow metastases.The diagnosis of gastric cancer was establishedexclusively through autopsy in this case. Noteworthy,retrospective analysis of the thorax roentgenogramallowed us an indirect evidence of the malignancy,based on normal heart silhouette associated to verysubtle opacities in medium lung fields, appearing asshort parallel horizontal lines extending to the pleuralsurfaces, resembling the Kerley B lines, as describedin typical cases of lung carcinomatous lymphangitis 1,11/14 (Fig. 5).159Budd-Chiari syndrome and gastric cancerThe widespread arterial tumor embolismof the lungs could explain the patient’s respiratorydistress. However, the absence of right ventricularhypertrophy-dilation, pulmonary hypertension orhemorrhagic infarcts, seems to emphasize theinfluence of lymphangitis as the cause of thepulmonary changes 14Lumbar pain and hematuria, in addition toincreased prothrombin time and high serumlevels of AP, AST, ALT, LDH and gGT 9 couldraise the clinical suspicion of Stauffer’s syndrome;however, autopsy findings of BCS and secondaryhepatic disruption may allow the exclusion of thathypothesis.Despite of respiratory support, intravenousfluids and red blood cells transfusion, the patientdeveloped refractory respiratory failure andirreversible circulatory shock and died in a shorttime. The intense thrombocytopenia and prolongedprothrombin time due to acute liver failure mayhave contributed to the gastrointestinal bleedingand hypovolemia. Since fibrin microthrombi werenot found in more than three different organs 4,the diagnosis of DIC was not established in thiscase in spite of thrombocytopenia and prolongedprothrombin time.Abdominal pain and hematemesis couldindicate the presence of gastric cancer; however,the rapid deterioration of clinical status hinderedthe gastroduodenal studies. Like in the vastmajority of cases, since diagnosis of BCS couldnot be established concomitant with early stagemalignancy, safe procedures would not beeffective for clinical management of the presenthepato-renal dysfunction7.Santos VM, Castro ECC, Araújo AC, Reis MA, Teixeira VPA. Síndrome de Budd-Chiari e adenocarcinomagástrico insuspeito.  Medicina (Ribeirão Preto) 2005; 38(2):  156-160.RESUMO: A síndrome de Budd-Chiari não tem sido descrita em pacientes jovens portadoresde câncer gástrico. Relata-se caso de síndrome de Budd-Chiari e linfangite carcinomatosa emhomem branco de 28 anos, portador de câncer gástrico insuspeito, apresentando icterícia,hematêmese e dispnéia. O estudo de autopsia revelou adenocarcinoma gástrico infiltrandovasos das camadas submucosa e serosa, com sangramento do estômago e intestino, além demetástases hepáticas e pulmonares. Múltiplos microtrombos mistos (fibrina, plaquetas e célu-las tumorais) foram observados em pequenos vasos sangüíneos pulmonares e tanto os vasoslinfáticos subpleurais quanto o interstício pulmonar continham células tumorais metastáticas.DESCRITORES: Adenocarcinoma. Gastrico. Linfangite Carcinomatosa. Síndrome de Budd-Chiari.  Síndrome de Stauffer. Trombose de Veia Hepática.Figure 5. Radiography of the thorax showing discrete opacities in lungfields, appearing as short parallel horizontal lines extending to the pleuralsurfaces, resembling the Kerley B lines.160Santos VM, Castro ECC, Araújo AC, Reis MA, Teixeira VPAREFERENCES1 - Bisetti A, Lalicata M, Jacot-des-Combes E. Adénocarcinomegastrique occulte avec lymphangite carcinomateusepulmonaire et anemie hémolityque microangiopathique chezun jeune adulte. Schweiz Med Wochenschr 1985;115: 561-4.2 - Colombo E, Giorgi S, Sonzini E, Velati C, Fenu P, Spinelli M.Disseminated intravascular coagulation and bone marrowmetastases as presenting manifestations of gastric carci-noma. Haematologica 1985; 70: 187.3 - Bresciani C, Borges PCM, Gama-Rodrigues JJ, Yamashita R,Marques CF, Strassmann V, Pinotti HW. Fatal pulmonary throm-boembolism in gastrectomy intraoperative procedures by gas-tric adenocarcinoma: Case report. Rev Hosp Clín Fac Med SPaulo 1999; 54: 115-20.4 - Santos VM, Rodrigues DBR, Castro ECC, Saldanha JC, SoaresS, Teixeira VPA, Reis MA. Widespread hematogenous me-tastases and Trousseau’s syndrome in gastric adenocarci-noma. Rev Hosp Clín Fac Med S Paulo 2001; 56: 91-6.5 - Crawford JM. The liver and the biliary tract. In: Cotran RS,Kumar V, Collins T, eds. Robbins Pathologic basis of disease.6th ed. Philadelphia:Saunders; 1999. p. 883.6 - Dilawari JB, Bambery P, Chawla Y, Kaur U, Bhusnurmath SR,Malhotra HS, Sood GK, Mitra SK, Khanna SK, Walia BS. He-patic outflow obstruction (Budd-Chiari syndrome). Experi-ence with 177 patients and review of the literature. Medicine1994; 73: 21-36.7 - Kakeji Y, Hashizume M, Kitano S, Hasuo K, Sugimachi K.Successful treatment of Budd-Chiari syndrome with earlygastric cancer. Int Surg 1991; 76: 55-7.8 - Takayasu K, Tajiri H, Noguchi M, Ishikawa T, Maruyama K.Imaging diagnosis of portal tumor thrombus secondary togastric cancer. Gastrointest Radiol 1989;14: 161-3.9 - Andrassy K, Gartner H, Siede WH, Ritz E, Riedasch G,Mohring K. Stauffer’s syndrome in renal cell carcinoma evi-dence for intravascular coagulation. Klin Wochenschr 1980;58: 91-7.10 - Fraisse TC, Damigny A, Di Castri A, De Wazieres B, FourcadeJ. Leiomyosarcoma and Stauffer syndrome. Rev Med In-terne 2001; 22: 1116-8.11 - Morillas MJ, Lopes J, Garcia-Cano J, Perez Vigara G, Pena J,Gonzalez Martin JA. Linfangitis carcinomatosa pulmonarcomo primera manifestación de cancer gástrico. Rev EspEnf Digest 1996; 88:379-80.12 - Aiello VD, Mansur AJ, Lopes EA, Bellotti G, Pileggi F. Severepulmonary hypertension due to carcinomatous lymphangitisof the lungs associated with unsuspected gastric cancer.Am Heart J 1988; 116: 197-8.13 - Hominal S, Falchero L, Perol M, Guerin JC. Carcinomatouslymphangitis. Presse Med 1999;28: 979-84.14 - Soares FA, Pinto AP, Landell GA , De Oliveira JA. Pulmonarytumor embolism to arterial vessels and carcinomatous lym-phangitis. A comparative clinicopathological study. Arch PatholLab Med 1993;117: 827-31.Recebido para publicação em 30/06/2004Aprovado para publicação em 01/06/2005

Síndrome de budd-chiari e adenocarcinoma gástrico insuspeito

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Budd-chiari syndrome and unsuspected gastric adenocarcinoma

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