RESUMO
OBJECTIVE: To present a rare entity of difficult preoperative diagnosis. BACKGROUND: Primary malignant melanoma of the esophagus represents 0.1% of all malignant tumors in this organ. It has been described in association with esophageal melanocytosis and melanosis with a clinic behavior similar to other neoplasm in the esophagus, but is more aggressive and fatal. By endoscopy, it usually appears as a vegetant, non-obstructive lesion, with dark pigmentation in the melanotic variant, the sole evidence that suggests the diagnosis prior to microscopic examination. Clinical exclusion of metastasis melanoma as a possibility is obligatory. METHODS: We reviewed clinical, imagenologic, endoscopic, and anatomopathological aspects of an ulcerovegetant neoplasm on the esophago-gastric junction, surgically resected, in a 65 years-old man with progressive dysphagia, who was without tumoral relapse during 18 months after surgery. RESULTS: The tumoral mass was an anaplastic cell tumor with very scant melanic pigmentation and diffuses stain for HMB-45 antigen and S100 protein, considering it to be a primary malignant melanoma. The neighboring mucous membrane showed a typical Barrett's esophagus and melanocytic hyperplasia. CONCLUSIONS: 1) Endoscopy may suggest the possibility of esophagus melanoma when there is a vegetate, non-obstructive, pigmented tumor. 2) Immunohistochemistry confirms the entity if HMB-45 antigen and S100 protein are detected in the tumoral cells, while the negative results for keratin and leukocytic common antigen (LCA) eliminate carcinoma and lymphoma as possible diagnoses. 3) In our case, the presence of Barrett's esophagus is exceptional, because it has not been described previously in the literature reviewed.
Assuntos
Esôfago de Barrett/complicações , Neoplasias Esofágicas/complicações , Melanoma/complicações , Idoso , Humanos , MasculinoRESUMO
We report a small, well-demarcated stromal tumor of the gallbladder in a 69-year-old woman. The tumor and associated cholelithiasis led to chronic cholecystitis symptoms. The wall of the gallbladder contained a 2.4-cm hypocellular nodule composed of bland spindle-shaped cells that were immunoreactive for vimentin, CD34, and CD117. With the latter antibody, which stains interstitial cells of Cajal (ICC), the neoplastic cells appear fusiform with elongated bipolar projections or dendritic-like cytoplasmic projections. The gallbladder wall adjacent to the tumor contained numerous CD117-positive cells in close contact with the normal smooth muscle cells, whereas two of 10 gallbladders with minimal chronic cholecystitis showed only a few CD117-positive cells. These findings provide evidence that this stromal tumor of the gallbladder shows ICC differentiation similar to some stromal tumors of the gut. The presence of numerous ICC in the uninvolved gallbladder wall suggests that this tumor might have evolved through hyperplasia of ICC.
Assuntos
Neoplasias da Vesícula Biliar/patologia , Plexo Mientérico/patologia , Neurilemoma/patologia , Células Estromais/patologia , Idoso , Antígenos CD34/análise , Biomarcadores Tumorais/análise , Colecistite/etiologia , Colecistite/patologia , Colelitíase/complicações , Colelitíase/patologia , Feminino , Neoplasias da Vesícula Biliar/química , Neoplasias da Vesícula Biliar/complicações , Neoplasias da Vesícula Biliar/cirurgia , Humanos , Técnicas Imunoenzimáticas , Plexo Mientérico/química , Proteínas de Neoplasias/análise , Neurilemoma/química , Neurilemoma/complicações , Neurilemoma/cirurgia , Proteínas Proto-Oncogênicas c-kit/análise , Células Estromais/química , Vimentina/análiseRESUMO
The present report describes opportunistic infections found at 74 autopsies of pediatric HIV/AIDS patients performed at several hospitals in Latin American countries. Fungal infections were the most common (53 cases), Candida sp. (39.18%) and Pneumocystis carinii (20.27%) being the most frequently recognized. Other fungal diseases included histoplasmosis, aspergillosis, and cryptococcosis. Viral infections were present in 31 cases, 38.7% being due to cytomegalovirus. Other viruses recognized included herpes simplex and adenovirus. Additional opportunistic infections were due to Mycobacterium avium-intracellulare, toxoplasmosis, and tuberculosis. Nonspecific bacterial bronchopneumonia was present in 11 cases. Cytomegalovirus and P. carinii coinfection was the most common association found. In this series patients died at a younger age (72% at or younger than 1 year old) and there was a slightly higher number of cases of histoplasmosis and brain toxoplasmosis than in other previously published series of infants and children.
Assuntos
Infecções Oportunistas Relacionadas com a AIDS/patologia , Infecções Oportunistas Relacionadas com a AIDS/epidemiologia , Adolescente , Animais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , América Latina/epidemiologia , MasculinoRESUMO
Fifty three pediatric patients with the histopathological diagnosis of lymphoblastic lymphoma (LL) were studied in a retrospective analysis during a 14 year period. Their age ranged from 1 to 16 years with a median of 7 years. Clinical staging was performed according to Murphy's system. There was one child in stage I (2%), 11 in stage II (21%), 14 stage III (26%) and 27 stage IV (51%). Patients in stage IV, 21 (78%) had initial bone marrow involvement, 4 (15%) central nervous system (CNS) infiltration and 2 (7%) simultaneous infiltration to the bone marrow and the CNS. The chemotherapy program consisted of induction, consolidation and maintenance with CNS prophylaxis. The whole program lasted 36 months. Out of 53 patients there were only 45 evaluable for treatment analysis response. A total of 14 (31%) are alive and in a continuous complete remission, with a median duration of remission of 66 months, 8 (18%) children abandoned treatment with a median duration of remission of 10 months. Twenty three patients (51%) are dead. The actuarial survival at 11 year is of 39% +/- 11% with a median remission rate for the whole group of 11.8 months. No patient in complete remission for more than 24 months has relapsed. We conclude that our chemotherapy program is more than adequate for early stages, but for advanced disease it has been a failure. There is a need to modify the chemotherapy program using a very similar protocol as the one used in high risk childhood acute lymphoblastic leukemia.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Análise Atuarial , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
We describe retrospectively the experience with 44 cases of AIDS from January 1987 to October 1991 at the Instituto Nacional de Pediatria, a tertiary care children hospital in Mexico City. All patients with 2 ELISA and a positive Western Blot test were included. Thirty three patients were infected perinatally (75%) and 11 through blood transfusion (25%). Fourty one patients belonged to the P2 classification of the Centers for Disease Control. Chronic diarrhea (77%), lymphadenopathy (75%), hepatomegaly/splenomegaly (70%) and oral candidiasis (61%) were the most common clinical findings. Twenty patients died (45.4%). No statistical relation were found between survival rate and the way of transmission and age at onset. Autopsy was performed in 14 patients and revealed a sharp decrease of lymphoid tissue at all levels with severe thymic atrophy.
Assuntos
Síndrome da Imunodeficiência Adquirida/epidemiologia , Síndrome da Imunodeficiência Adquirida/fisiopatologia , Criança , Pré-Escolar , Feminino , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Masculino , México/epidemiologia , Estudos Retrospectivos , Análise de SobrevidaRESUMO
Over one hundred patients diagnosed with vascular purpura at the National Institute of Pediatrics between 1979 and 1988 were retrospectively studied. Frequency was similar for both sexes. 82% were between the ages of six months and 11 years old. The extrarenal clinical manifestations were: petechias in 98%; abdominal pain 78% arthralgias 45%; melena 39%; and arthritis in 19% of the cases, 49 patients had nephropathy of different degrees and they did differently, 32 had hematuria with or without proteinuria within a nephrotic range, seven suffered from a nephrotic syndrome, one with a nephritic syndrome and nine others had a combination of two or three syndromes (nephritic/nephrotic/renal failure) with an unfavorable evolution towards terminal renal failure in eight of them; on the other hand, this was not seen in the remaining patients. Thirteen renal biopsies were taken from patients with more severe clinical manifestations, finding in them mesangial proliferation or endo- and extracapillary proliferation. These findings suggest that the initial clinical presentation of the illness allows for the prediction of the future.
Assuntos
Vasculite por IgA/complicações , Nefropatias/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Vasculite por IgA/epidemiologia , Lactente , Nefropatias/epidemiologia , Nefropatias/etiologia , Masculino , México/epidemiologia , Nefrite/diagnóstico , Nefrite/epidemiologia , Nefrite/etiologia , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/epidemiologia , Síndrome Nefrótica/etiologia , Prognóstico , Estudos RetrospectivosRESUMO
Vascular congenital abnormalities are common in children and are often seen in skin and soft tissues. They are rarely observed in internal organs. They may be found singly or in a multiple fashion. The liver is the most frequently affected organ by this disease. The most common histological variety is the hemangioendothelioma. This is a rare neoplasm of the pancreas. The case of a six month old boy with a pancreatic head hemangioendothelioma, associated with thrombocytopenia and digestive track bleeding is presented. The case is clinically interesting due to the vascular malformation and its location. These in turn caused obstructive neonatal jaundice and very severe liver lesions which contributed to the child's death. Paradoxically, the neoplasm is not considered malignant and the secondarily produced liver lesions could have been prevented if the patient would have been diagnosed correctly and at an earlier stage. Hemangioendothelioma; Kasabach-Merrit syndrome; neonatal cholestatic syndrome.
Assuntos
Colestase/etiologia , Neoplasias do Ducto Colédoco/complicações , Hemorragia Gastrointestinal/etiologia , Hemangioendotelioma/complicações , Neoplasias Pancreáticas/complicações , Trombocitopenia/etiologia , Neoplasias do Ducto Colédoco/patologia , Hemangioendotelioma/patologia , Humanos , Lactente , Masculino , Neoplasias Pancreáticas/patologia , SíndromeRESUMO
The clinical, histopathologic, and mycologic aspects of a case of subcutaneous phycomycosis caused by Basidiobolus haptosporus in a 3-year-old boy are reported. This is the first documented case from Central and North America. Oral potassium iodide and trimethoprim-sulfamethoxazole gave excellent therapeutic results.