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1.
Gan To Kagaku Ryoho ; 47(7): 1121-1123, 2020 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-32668866

RESUMO

A 60-year-old Khmer woman visited a hospital established by a Japanese non-profit organization in the Kingdom of Cambodia with complaints of swelling in the left abdomen and appetite loss for 2 months. Abdominal computed tomography scan showed a mass measuring 14.6×13.4×19.3 cm with internal necrosis in the left abdomen. On laparotomy, a large tumor involving the jejunum had adhered to the transverse and descending colons. The tumor, measuring 25×20×10 cm and weighing 2,994 g, was excised along with 65 cm of the jejunum. Histopathological examination revealed a gastrointestinal stromal tumor(GIST). Postoperative course was uneventful. Thanks to the cooperation with the Japanese and the Cambodian people, the surgery was successful in spite of a shortage of medical personnel and medical resources in Cambodia.


Assuntos
Tumores do Estroma Gastrointestinal , Neoplasias do Jejuno , Camboja , Feminino , Humanos , Jejuno , Laparotomia , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
2.
Gan To Kagaku Ryoho ; 47(1): 180-182, 2020 Jan.
Artigo em Japonês | MEDLINE | ID: mdl-32381898

RESUMO

A 71-year-old male presented with abdominal distension and fever to our hospital. Abdominal CT revealed a huge tumor in abdomen, and non-curative surgery was performed. Peritoneal dissemination was widespread and the tumor invaded the bladder and sigmoid-colon mesenterium. Two months after the initial surgery, CT showed liver metastasis, and oral administration of imatinib mesylate was started. The peritoneal dissemination and liver metastasis showed a decrease, and this was well controlled for 45 months without severe side effects. Abdominal CT revealed peritoneal dissemination in the ileocecum after 43 months since the administration of imatinib. Therefore, sunitinib treatment was initiated. After 3 months of sunitinib administration, the tumor perforated. Emergency operation was performed to resect the ileocecum, and sunitinib was continued for 1 year. In GIST with liver metastasis and peritoneal dissemination, repeated surgical resection combined with chemotherapy is important to improve the patient's survival.


Assuntos
Antineoplásicos/uso terapêutico , Tumores do Estroma Gastrointestinal/tratamento farmacológico , Mesilato de Imatinib/uso terapêutico , Neoplasias do Jejuno/tratamento farmacológico , Neoplasias Hepáticas , Idoso , Tumores do Estroma Gastrointestinal/secundário , Humanos , Jejuno , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/secundário , Masculino
3.
J Surg Res ; 252: 116-124, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32278965

RESUMO

BACKGROUND: Proximal (duodenal) small bowel adenocarcinomas have a worse prognosis than distal (jejuno-ileal) tumors, but differences in patient, tumor, and treatment factors between locations remain unclear. METHODS: Patients in the National Cancer Database with surgically resected pathologic stage I-IV small bowel adenocarcinomas between 2004 and 2015 were analyzed. Clinical stage IV patients were excluded. RESULTS: Proximal tumors (n = 3767) were more likely to be higher grade (OR 1.52, CI 1.22-1.85 for moderately; OR 1.83, CI 1.49-2.33 for poorly differentiated, P < 0.01 for both) and have positive lymph nodes (OR 2.04, CI 1.30-3.23, P < 0.01), while distal tumors (n = 3252) were likely to be larger (OR 1.31, CI 1.07-1.60 for size > 5 cm, P < 0.01). Proximal tumors were associated with worse overall survival (OS) and stage-specific survival compared with distal tumors (all P < 0.01). Cox regression analysis of the entire cohort showed worse survival with community versus academic cancer programs, higher comorbidity scores, pathologic stage IV, poorly differentiated histology, positive nodal or margin status, and proximal location, while female gender, larger tumor size, and chemotherapy predicted better survival. On separate Cox regression analyses of each location, neoadjuvant chemotherapy was associated with better OS in the proximal cohort (HR 0.70, CI 0.55-0.88, P < 0.01), while adjuvant chemotherapy was associated with better OS for both proximal (HR 0.49, CI 0.42-0.57, P < 0.01) and distal tumors (HR 0.68, CI 0.57-0.81, P < 0.01). CONCLUSIONS: Proximal small bowel adenocarcinomas are associated with worse overall and stage-specific survival. This may be due to tumor biologic differences as proximal tumors were more likely to have higher grade. Future studies should further investigate differences between proximal and distal tumors to guide targeted treatment algorithms.


Assuntos
Adenocarcinoma/mortalidade , Neoplasias Duodenais/mortalidade , Neoplasias do Íleo/mortalidade , Neoplasias do Jejuno/mortalidade , Adenocarcinoma/patologia , Adenocarcinoma/terapia , Idoso , Quimiorradioterapia Adjuvante , Neoplasias Duodenais/patologia , Neoplasias Duodenais/terapia , Duodeno/patologia , Duodeno/cirurgia , Feminino , Humanos , Neoplasias do Íleo/patologia , Neoplasias do Íleo/terapia , Íleo/patologia , Íleo/cirurgia , Neoplasias do Jejuno/patologia , Neoplasias do Jejuno/terapia , Jejuno/patologia , Jejuno/cirurgia , Estimativa de Kaplan-Meier , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Terapia Neoadjuvante , Gradação de Tumores , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Taxa de Sobrevida , Resultado do Tratamento
6.
Medicine (Baltimore) ; 99(4): e18863, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977886

RESUMO

RATIONALE: Hemolymphangioma is a benign tumor comprised of the newly-formed lymph spaces and blood vessels, which can usually be found in the head and neck of the affected children. There are few reports regarding cases with hemolymphangioma in small intestine, spleen, esophagus, and other organs. PATIENT CONCERNS: Herein, a 55-year-old woman was presented in this study, she had complained of discomfort in the right upper abdomen for 2 months, and was discovered with a space-occupying lesion in proximal jejunum on computed tomography (CT). Eventually, the lesions were confirmed through double-balloon enteroscopy (DBE) to be located in the jejunum 60 cm away from the Treitz ligament. DIAGNOSE: Subsequently, the small intestine was partially resected, and postoperative pathology had confirmed the diagnosis of small intestinal hemolymphangioma. INTERVENTIONS: Excisional surgery of the lesion was planned. On surgery, the lesions were discovered to be about 33 cm to 22 cm when engorged the superficial vessels. No enlarged lymph nodes were seen at the root of the mesentery, and no obvious lesion was observed in the remaining small intestine. OUTCOMES: Follow-up for 6 months showed no recurrence. LESSONS: Hemolymphangioma lacks typical clinical symptoms, and the correct preoperative diagnosis of hemolymphangioma remains challenging. Due to the increasing use of endoscopic diagnostic techniques, it is expected that hemolymphangioma in gastrointestinal tract may be detected and endoluminal located before surgery more feasibly. This case report aimed to highlight the contributions of CT and DBE to an accurate preoperative diagnosis and surgical strategy planning.


Assuntos
Neoplasias do Jejuno/diagnóstico por imagem , Neoplasias do Jejuno/cirurgia , Linfangioma/diagnóstico por imagem , Linfangioma/cirurgia , Enteroscopia de Duplo Balão , Feminino , Humanos , Neoplasias do Jejuno/patologia , Linfangioma/patologia , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
8.
BMC Surg ; 19(1): 194, 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31842856

RESUMO

BACKGROUND: Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications. CASE PRESENTATION: We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis. CONCLUSIONS: Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation.


Assuntos
Apendicite/diagnóstico , Fibromatose Agressiva/diagnóstico , Neoplasias do Jejuno/diagnóstico , Abdome Agudo/etiologia , Doença Aguda , Adulto , Diagnóstico Diferencial , Fibromatose Agressiva/complicações , Fibromatose Agressiva/cirurgia , Humanos , Neoplasias do Jejuno/complicações , Neoplasias do Jejuno/cirurgia , Masculino , Peritonite/etiologia
9.
Medicine (Baltimore) ; 98(51): e14222, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31860943

RESUMO

RATIONALE: Patients with gastrointestinal stromal tumors (GISTs) are often found to have liver metastases at their 1st presentation. Most patients need preoperative treatment to reduce the size of the liver metastases to increase the possibility of surgical resection. Currently, imatinib mesylate is the drug of 1st choice for preoperative treatment and sunitinib malate (SM) is seldom used. Here we report a case of GIST with liver metastases where SM was used as a preoperative treatment. PATIENT CONCERNS: A 56-year-old worker presented with intermittent abdominal pain and eating difficulties. DIAGNOSES: An enhanced computed tomography scan showed a 15 × 15 × 10 cm malignant mass in the upper abdomen, and 2 metastases (15.1 × 13.1 cm and 14.8 × 8.8 cm) in the liver. The postcaval and middle hepatic veins were compressed by the liver metastases, making radical resection very difficult. INTERVENTIONS: First the primary tumor in the jejunum was resected, and then SM was used as a preoperative treatment to reduce the size of the liver metastases to improve the possibility of surgical resection. OUTCOMES: Both liver metastases regressed considerably in size and it was then possible to perform a radical resection. LESSONS: The SM has the potential to be used as preoperative therapy for GIST with large liver metastases. This method provides a new option for the preoperative treatment of GIST with liver metastases.


Assuntos
Tumores do Estroma Gastrointestinal/tratamento farmacológico , Tumores do Estroma Gastrointestinal/patologia , Neoplasias do Jejuno/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/secundário , Sunitinibe/uso terapêutico , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Seguimentos , Tumores do Estroma Gastrointestinal/cirurgia , Hepatectomia/métodos , Humanos , Neoplasias do Jejuno/patologia , Neoplasias do Jejuno/cirurgia , Neoplasias Hepáticas/cirurgia , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante/métodos , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Cuidados Pré-Operatórios/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
10.
J. coloproctol. (Rio J., Impr.) ; 39(4): 385-388, Oct.-Dec. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-1056637

RESUMO

Abstract Gastrointestinal stromal tumors, although rare, are the most common primary mesenchymal neoplasms of the gastrointestinal tract and originate from the interstitial cells of Cajal. They present slow growth and symptoms such as bleeding, abdominal pain or discomfort, and the presence of an abdominal mass. The most affected organs are the stomach and small intestine. Differential diagnoses for gastrointestinal stromal tumor include adenocarcinoma and small intestine lymphoma, metastasis, and carcinoid tumor. Gastrointestinal stromal tumors have been associated with familial syndromes such as type 1 neurofibromatosis, considered a predisposing factor for tumors in the small intestine. This study aimed to report a case of gastrointestinal stromal tumor in the jejunal region in a patient with type 1 neurofibromatosis, followed-up for two years, who underwent laparoscopic segmental enterectomy and diagnosis determined by histopathology and immunohistochemistry. The diagnosis of small intestine gastrointestinal stromal tumor is challenging because of its low incidence, nonspecific symptoms, relative inaccessibility of the small intestine to conventional endoscopic examination, broad spectrum of radiological appearances, and the fact that the nature of the mass is difficult to determine with imaging examinations of the abdomen alone. Thus, the small intestine gastrointestinal stromal tumor may be erroneously diagnosed as pancreatic, gynecological, or mesenteric tumors. The literature does not present many reports on the association of jejunal gastrointestinal stromal tumor with neurofibromatosis. Understanding the tumoral behavior of small intestine gastrointestinal stromal tumor in this subgroup of patients would allow better follow-up.


Resumo Os tumores estromais gastrointestinais, embora raros, são as neoplasias mesenquimais primárias mais comuns do trato gastrointestinal e originam-se das células intersticiais de Cajal. Apresentam crescimento lento e manifestam sintomas como sangramento, dor ou desconforto abdominal e presença de massa abdominal. Os órgãos mais acometidos são estômago e intestino delgado. Os diagnósticos diferenciais para tumores estromais gastrointestinais incluem adenocarcinoma e linfoma de intestino delgado, metástases e tumor carcinoide. Os tumores estromais gastrointestinais têm sido associados a síndromes familiares como a neurofibromatose tipo 1, considerada um fator predisponente para tumores no intestino delgado. O objetivo desse trabalho é relatar um caso de tumor estromal gastrointestinal em região jejunal em paciente portadora de neurofibromatose tipo 1, com 2 anos de seguimento, submetida a enterectomia segmentar laparoscópica e diagnóstico determinados pela histopatologia e imuno-histoquímica. O diagnóstico de tumor estromal gastrointestinal do intestino delgado é desafiador, devido a sua baixa incidência, sintomas inespecíficos, relativa inacessibilidade do intestino delgado ao exame endoscópico convencional, amplo espectro de aparências radiológicas e difícil determinação da natureza da massa apenas com exames de imagens do abdome. Assim, tumor estromal gastrointestinal no intestino delgado podem ser erroneamente diagnosticados como tumores pancreáticos, tumores ginecológicos, ou tumores do mesentério. A descrição científica da associação de tumor estromal gastrointestinal de jejuno com neurofibromatose é incomum. Tais descrições permitem melhor seguimento dos pacientes a partir do momento que se entende o comportamento tumoral do tumor estromal gastrointestinal de intestino delgado nesse subgrupo de pacientes.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Neurofibromatose 1/complicações , Tumores do Estroma Gastrointestinal/complicações , Neoplasias do Jejuno/complicações , Laparoscopia , Tumores do Estroma Gastrointestinal/cirurgia , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Neoplasias do Jejuno/cirurgia , Neoplasias do Jejuno/diagnóstico por imagem
11.
Pan Afr Med J ; 33: 197, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31692688

RESUMO

Gastrointestinal bleedings caused by small intestinal tumors are rare and difficult to diagnose because they are not easy to access to the conventional endoscopy. We report two cases, one of them from proximal jejunum and the other one from ileal intestine complicated by intussusception. The two cases were admitted in the emergency department for hematochezia and melena, the diagnosis was established by enhanced helical computed tomography angiography.


Assuntos
Hemorragia Gastrointestinal/etiologia , Neoplasias do Íleo/complicações , Neoplasias do Jejuno/complicações , Idoso , Angiografia por Tomografia Computadorizada , Hemorragia Gastrointestinal/diagnóstico , Humanos , Neoplasias do Íleo/diagnóstico , Intussuscepção/complicações , Intussuscepção/diagnóstico , Neoplasias do Jejuno/diagnóstico , Masculino
12.
Pan Afr Med J ; 33: 214, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31692747

RESUMO

Primary melanoma of the small bowel is a rare clinical entity with a paucity of published reports in literature. Most cases of gastrointestinal melanomas are metastatic lesions arising from skin or ocular origins. This is a case report of a 63 year old female with adult intussusception with jejunal melanoma as the lead point. The index patient had a long history of abdominal pain associated with significant weight loss and presented with features of intestinal obstruction. The possibility of a regressed or unidentified extra-intestinal site cannot be absolutely excluded as the patient did not have a PET scan. Due to the vague nature of clinical symptoms and signs, the diagnosis of small bowel melanoma is difficult, especially in patients with no obvious cutaneous pathology. A high index of suspicion for melanoma as a malignant lead point for adult intussusception should always be entertained.


Assuntos
Intussuscepção/etiologia , Neoplasias do Jejuno/diagnóstico , Melanoma/diagnóstico , Dor Abdominal/etiologia , Feminino , Humanos , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/etiologia , Intussuscepção/diagnóstico , Neoplasias do Jejuno/complicações , Melanoma/complicações , Pessoa de Meia-Idade
13.
BMJ Case Rep ; 12(11)2019 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-31767604

RESUMO

Mesenteric lymphangiomas are relatively rare, with clinical symptoms ranging from an asymptomatic presentation to an acute abdomen. The natural history and biological behaviour of this entity can range from slow indolent lesions to aggressive tumours with a risk of malignant transformation. Spontaneous regression of a mesenteric lymphangioma is rare. We herein report a case of a jejunal mesenteric lymphangioma that was initially detected incidentally in an asymptomatic patient with a subsequent sudden increase in size with resulting surrounding mass effect after 9 months and a spontaneous partial regression at surgical resection. Our case is the first reported case which outlines a period in the natural history and evolution of microcystic mesenteric lymphangioma, illustrating the sudden enlargement likely attributable to spontaneous and self-limiting haemorrhage and subsequent partial regression at surgical resection. We thenceforth propose a possible management algorithm for adult patients with mesenteric lymphangiomas.


Assuntos
Neoplasias do Jejuno/patologia , Linfangioma Cístico/patologia , Regressão Neoplásica Espontânea/patologia , Neoplasias Peritoneais/patologia , Idoso , Algoritmos , Gerenciamento Clínico , Humanos , Neoplasias do Jejuno/terapia , Jejuno/patologia , Linfangioma Cístico/terapia , Masculino , Mesentério/patologia , Neoplasias Peritoneais/terapia
14.
World J Surg Oncol ; 17(1): 170, 2019 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-31651341

RESUMO

BACKGROUND: Lymphangiomas are uncommon congenital malformations that present mainly in the head, neck, and axillar regions in pediatric patients. Mesenteric cystic lymphangiomas (MCLs), which occasionally present with substantial growth and the invasion of adjacent vital structures, are rarely reported in adults. We report a case of MCL in an adult who was treated with laparoscopic-assisted excision. CASE PRESENTATION: A 40-year-old Japanese man visited his family physician for prolonged periumbilical pain. Plain computed tomography (CT) showed a low-density mass in his left abdomen, and he was referred to our hospital 2 weeks later. His abdomen was flat and soft, and no mass was felt upon palpation. Routine laboratory data showed no abnormalities in the blood cell counts. The levels of tumor markers, such as carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125), were within normal ranges. Contrast-enhanced CT was performed, and a low-density mass was observed with an irregular outline and poor contrast, as well as involvement of the peripheral mesenteric artery and partial compression of the adjacent jejunum without dilatation of the oral side of the bowel. The patient was diagnosed with lymphatic cysts and observed for 1 month without symptom exacerbation. Follow-up CT showed no increase in the size of the mass but showed apparent invasion of the jejunal wall without bowel obstruction. Magnetic resonance imaging (MRI) showed intermediate intensity on T1-weighted imaging (T1WI) and high intensity on T2-weighted imaging (T2WI). The coronal view on T2WI clearly showed an accumulation of cystic lesions. We performed tumor excision with partial resection of the jejunum in a laparoscopic-assisted manner. Pathological examination showed multicystic lesions with an attenuated endothelial lining, surrounding rich adipose tissue and scattered smooth muscle fibers; the patient was diagnosed with MCL. Immunohistochemical assays supported this diagnosis. CONCLUSIONS: This is rare case of MCL presenting in an adult who underwent successful laparoscopic-assisted resection. Mesenteric lymphangioma (ML) should be considered in the differential diagnosis of patients with intraabdominal cysts. Radical excision is optimal, even when the patient is asymptomatic. Laparoscopic-assisted tumor resection is a suitable surgical method for treating MLs located in the peripheral mesentery.


Assuntos
Neoplasias do Jejuno/cirurgia , Laparoscopia/métodos , Linfangioma Cístico/cirurgia , Neoplasias Peritoneais/cirurgia , Adulto , Humanos , Neoplasias do Jejuno/diagnóstico por imagem , Neoplasias do Jejuno/patologia , Linfangioma Cístico/diagnóstico por imagem , Linfangioma Cístico/patologia , Masculino , Mesentério , Neoplasias Peritoneais/diagnóstico por imagem , Neoplasias Peritoneais/patologia , Tomografia Computadorizada por Raios X
15.
Dtsch Med Wochenschr ; 144(21): 1505-1508, 2019 10.
Artigo em Alemão | MEDLINE | ID: mdl-31634928

RESUMO

HISTOLOGY AND CLINICAL FINDINGS: We report on a 66-year-old-patient with plasmocytoma, who presented with melena since 3 days, dyspnea and dizzyness. INVESTIGATION AND DIAGNOSIS: Because of the laboratory and clinical examination the suspected diagnosis was an active gastrointestinal bleeding. The performed gastroduodenoscopy and sigmoidoscopy could not verify an active bleeding. Thus a contrast-enhanced computed tomography was performed additionally and showed abnormal enhancement as well as a ballooning of the proximal jejunum. Subsequently a jejunoscopy was performed where an actively bleeding mass was found. TREATMENT AND COURSE: Due to the known medical history of a plasmocytoma and in conjunction with the performed examinations the suspected diagnosis of an extramedullary metastasis of a plasmocytoma in the jejunum was made. Since only temporary control of the bleeding could be achieved, a segmental resection of the small bowel was performed. The following histological examination confirmed the diagnosis. CONCLUSION: The differential diagnosis for gastrointestinal bleeding includes also rarely neoplastic manifestations in the small bowel. Contrast-enhanced computed tomography can aide in the localization of the bleeding side and determination of the cause of hemorrhage.


Assuntos
Neoplasias do Jejuno , Melena/etiologia , Plasmocitoma , Idoso , Endoscopia Gastrointestinal , Feminino , Humanos , Neoplasias do Jejuno/complicações , Neoplasias do Jejuno/diagnóstico por imagem , Neoplasias do Jejuno/cirurgia , Jejuno/diagnóstico por imagem , Jejuno/cirurgia , Plasmocitoma/complicações , Plasmocitoma/patologia
17.
Nihon Shokakibyo Gakkai Zasshi ; 116(10): 826-832, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31597881

RESUMO

A 75-year-old woman presented complaining of anorexia. A malignant gastrointestinal lymphoma was diagnosed, and chemotherapy was initiated. After 2 months, she developed vomiting. Computed tomography (CT) revealed thickening of the jejunal wall and dilatation of the intestine proximal to that area. Positron emission tomography-CT showed no uptake. Small bowel stenosis due to cicatricial stenosis after chemotherapy was suspected. Laparoscopic partial resection of the stenotic small bowel segment was performed. Histopathologically, only granulation tissue was seen with no evidence of tumor. Occasionally, cicatricial stenosis can develop after chemotherapy for malignant gastrointestinal lymphoma. Therefore, this condition must be considered an important complication of treatment for this disease.


Assuntos
Obstrução Intestinal , Neoplasias do Jejuno , Linfoma , Idoso , Constrição Patológica , Feminino , Humanos , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons
18.
G Chir ; 40(3): 225-229, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31484013

RESUMO

INTRODUCTION: Jejunal adenocarcinoma is a very rare disease but the frequency of this rare carcinoma is higher in celiac patients. We report the first case report of a second jejunal loop adenocarcinoma associated with celiac disease. PRESENTATION OF CASE: A 47-year-old woman, with a history of celiac disease. Computerized tomographic scans of the abdomen and pelvis demonstrated a severe retroperitoneal lymphoadenopathy, para-aortic, inter-aorto-caval, porto-caval, posterior pancreaticoduodenal space, celiac trunk, lesser gastric curvature, lymph node grouping. The patient underwent digiunal resection and regional lymphadenectomy. Diagnosis was poorly differentiated jejunal adenocarcinoma, infiltrating subserosal adipose tissue, metastasing in five out of eight regional lymph nodes. U.I.C.C. 2017 grading = pT3 pN2 G3 R0; Stage IIIB. DISCUSSION: The jejunum accounts for 11-25% of small bowel adenocarcinoma, that accounts for less than 5% of gastrointestinal cancer, notwithstanding that 90% of the mucosa surface area of the digestive tract is made by small intestine. To the best of our knowledge, this is the first report on a second loop jejunal adenocarcinoma complicating celiac disease. In our study, the diagnosis of cancer was made by computed tomography (CT) of abdomen and the patient was operated. For the diagnosis of small bowel tumour, CT enteroclysis has a sensitivity of 85-95% and a specificity of 90-96%. Complete resection (RO) of the jejunal adenocarcinoma, with regional lymph nodes resection and jejuno-jejunal anastomosis should be performed. CONCLUSION: After curative surgical resections of small bowel adenocarcinoma, adjuvant chemo-therapy has not shown a clear benefit in retrospective studies. Preoperative Chemo-Radio-therapy and careful Imaging Staging are the first steps to planning surgery.


Assuntos
Adenocarcinoma/complicações , Doença Celíaca/complicações , Neoplasias do Jejuno/complicações , Doenças Raras/complicações , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/cirurgia , Feminino , Humanos , Neoplasias do Jejuno/diagnóstico por imagem , Neoplasias do Jejuno/cirurgia , Jejuno/diagnóstico por imagem , Jejuno/cirurgia , Excisão de Linfonodo , Pessoa de Meia-Idade , Doenças Raras/diagnóstico por imagem , Doenças Raras/cirurgia , Tomografia Computadorizada por Raios X
19.
Ann Hematol ; 98(11): 2541-2550, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31493002

RESUMO

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a provisional entity in the 2017 World Health Organization classifications. To further elucidate the clinicopathologic features of this new disease, we carried out a retrospective, multicenter analysis of 42 patients with MEITL. The median age of the patients was 59 years (range, 20-84 years), and 27 patients (64 %) were male. Thirty-two patients (76 %) were Ann-Arbor stages I-II and 28 (67 %) were Lugano stages I-II1&2. The most frequent site of involvement was the jejunum (N = 21). Most cases expressed CD8 (79 %) and CD56 (95 %) and did not express CD30 (5 %) or EBER (0 %). The median progression-free survival was 6.9 months (95 % CI 4.3-9.6); the median OS was 14.8 months (2.4-27.2). Thirty-two patients (76 %) underwent surgery and 37 (88 %) received chemotherapy. A complete response (CR) rate was 38 %. Sixteen patients had undergone autologous stem cell transplantation (ASCT). Relapse or progression was documented in 24 cases, most frequently in the primary site (N = 23). Four cases showed central nervous system relapse. Age over 55 years, poor performance scale, advanced Lugano stage (IIE-IV), not achieving CR, and not receiving ASCT were associated with inferior OS. While the optimal management of MEITL remains undetermined, achieving CR and consolidative ASCT seem essential. As CHOP might be insufficient for achieving CR, more efficient combinations should be investigated. Additionally, considering the frequent local failure and CNS relapse, novel therapeutic approaches are required to improve survival.


Assuntos
Antígenos CD/biossíntese , Neoplasias do Jejuno , Linfoma de Células T Periférico , Proteínas de Neoplasias/biossíntese , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Humanos , Neoplasias do Jejuno/metabolismo , Neoplasias do Jejuno/mortalidade , Neoplasias do Jejuno/patologia , Neoplasias do Jejuno/terapia , Linfoma de Células T Periférico/metabolismo , Linfoma de Células T Periférico/mortalidade , Linfoma de Células T Periférico/patologia , Linfoma de Células T Periférico/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
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