ABSTRACT
Histiocytic sarcoma (HS) is a rare malignant neoplasia of hematopoietic origin and unknown etiology. We studied three patients with histiocytic sarcoma reviewing the morphological and immunohistochemical aspects. We evaluated in particular, if apoptosis may be unbalanced in this disease. All cases have morphological and immunohistochemical features consistent with the diagnosis of histiocytic sarcoma. The markers CD163, CD68, vimentin, lysozyme, and S-100 were positive in all cases. Similarly, the three samples were positive for Fas-ligand and Caspase-3. It is well-known that neoplasms may induce increased levels of Fas-ligand with the blockade of the apoptosis process. In the context of HS, the increased Fas-ligand expression represents a new area for research. Indeed, it is linked to proinflammatory stimulus and, maybe with the association of an infection.
Subject(s)
Humans , Male , Female , Adult , Aged , Histiocytic Sarcoma/etiology , Apoptosis , Caspase 3 , Fas Ligand Protein , Histiocytic Sarcoma/diagnosis , ImmunohistochemistryABSTRACT
Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period.
Sarcoma histiocítico é uma rara entidade neoplásica maligna hematológica originada de clones celulares dendríticos ou histiocitários. As lesões podem ser nodais ou extranodais, mais comumente no trato gastrintestinal. Um pequeno número de casos apresenta lesões cutâneas exclusivas. O diagnóstico definitivo é feito pela positividade dos marcadores imunohistoquímicos CD163, CD68, CD4 e lisozima. O tratamento é controverso, geralmente com quimioterapia combinada sistêmica. Este é um caso de sarcoma histiocítico cutâneo em uma paciente de 82 anos apresentando duas lesões nodulares na mama e braço direitos, tratadas com exérese simples e seguimento multidisciplinar, evitando-se condutas intempestivas e investigações exaustivas. Apesar de a maioria dos estudos relatar evolução agressiva, a paciente apresenta quadro clínico bom e estável no período de doze meses de seguimento.
Subject(s)
Aged, 80 and over , Humans , Male , Histiocytic Sarcoma/diagnosis , Skin Neoplasms/diagnosis , Biomarkers/analysis , Follow-Up Studies , Histiocytic Sarcoma/surgery , Immunohistochemistry , Skin Neoplasms/surgery , Treatment OutcomeABSTRACT
We report a rare case of extranodal histiocytic sarcoma with multifocal gastrointestinal tract involvement, which has not been documented in the literature so far. A diagnosis of interdigitating dendritic cell/ histiocytic sarcoma was made on the preoperative gastric biopsy. Computed tomography scan revealed multifocal, circumferential gastrointestinal wall thickening involving the stomach and jejunal loops. Patient underwent distal gastrectomy with extended D1 dissection and proximal jejunal resection with gastrojejunostomy. Immunohistochemistry profile of both the gastric and jejunal tumors was similar to the preoperative gastric biopsy. The histiocytic origin of the tumor was confirmed by positive reaction of the tumor cells for CD 163. She received four cycles of CHOP chemotherapy, and is free of disease three years, following surgery.
Subject(s)
Adult , Antigens, CD/analysis , Antigens, Differentiation, Myelomonocytic/analysis , Biopsy , Female , Gastrectomy , Gastric Bypass , Gastrointestinal Neoplasms/diagnosis , Gastrointestinal Neoplasms/pathology , Gastrointestinal Neoplasms/surgery , Histiocytic Sarcoma/diagnosis , Histiocytic Sarcoma/pathology , Histiocytic Sarcoma/surgery , Histocytochemistry , Humans , Immunohistochemistry , Jejunum/pathology , Microscopy , Receptors, Cell Surface/analysis , Stomach/pathology , Tomography, X-Ray ComputedABSTRACT
A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.
Subject(s)
Humans , Female , Middle Aged , Neoplasms , Histiocytic Sarcoma/diagnosisABSTRACT
Histiocytic sarcoma is a rare malignant neoplasm that originates from a histiocytic hematopoietic lineage characterized by histiocytic differentiation and its corresponding immunophenotypic features. Patients with histiocytic sarcoma usually have a poor prognosis due to its aggressive clinical behavior. Here we report a rare case of extranodal histiocytic sarcoma of the stomach which was confirmed through immunohistochemical staining. A 71-yearold man was presented with epigastric pain. Gastroscopy, abdominal CT, and EUS revealed a mass located on the posterior wall of upper body and fundus of the stomach. Grossly, grayish white solid masses were seen extending down to the submucosal layer. Microscopically, the tumor cells had eosinophilic cytoplasm, abundant vacuole, and mitosis. Immunohistochemical staining revealed that the tumor cells were positive for LCA, CD68, and lysozyme. Early detection and accurate diagnosis of this rare neoplasm is important because it can make a great difference in prognostic outcomes. To make an accurate and definitive diagnosis, immunohistochemical staining is essential in the confimation of histiocytic orign.
Subject(s)
Aged , Humans , Male , Adenocarcinoma/diagnosis , Antigens, CD/metabolism , Leukocyte Common Antigens/metabolism , Antigens, Differentiation, Myelomonocytic/metabolism , Diagnosis, Differential , Gastroscopy , Histiocytic Sarcoma/diagnosis , Muramidase/metabolism , Stomach Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
Histiocytic sarcoma (HS) is a very rare neoplasm that often shows an aggressive clinical course and systemic symptoms, such as fever, weight loss, adenopathy, hepatosplenomegaly and pancytopenia. It may present as localized or disseminated disease. We describe here a 63-yr-old male who manifested systemic symptoms, including fever, weight loss and generalized weakness. Abdominal and chest computed tomography failed to show specific findings, but there was suspicion of multiple bony changes at the lumbar spine. Fusion whole body positron emission tomography, bone scan and lumbar spine magnetic resonance imaging showed multiple bone lesions, suggesting a malignancy involving the bone marrow (BM). Several BM and bone biopsies were inconclusive for diagnosis. Necropsy showed replacement of the BM by a diffuse proliferation of neoplastic cells with markedly increased cellularity (95%). The neoplastic cells were positive for lysozyme and CD68, but negative for T- and B-cell lineage markers, and megakaryocytic, epithelial, muscular and melanocytic markers. Morphologic findings also distinguished it from other dendritic cell neoplasms.
Subject(s)
Humans , Male , Antigens, CD/metabolism , Antigens, Differentiation, Myelomonocytic/metabolism , Bone Marrow/metabolism , Bone Marrow Neoplasms/diagnosis , Diagnosis, Differential , Histiocytic Sarcoma/diagnosis , Magnetic Resonance Imaging , Muramidase/metabolism , Positron-Emission Tomography , Tomography, X-Ray ComputedABSTRACT
Histiocytic sarcoma is a malignant proliferation of cells showing morphologic and immunophenotypic features similar to those of mature tissue histiocytes and is known for its rapid progression and poor prognosis. We describe a case of histiocytic sarcoma diagnosed by bone marrow biopsy. A 64-yr-old male was admitted for fever and weight loss that persisted for 8 months. The patient died undiagnosed on the 7th hospitalization day. A bone marrow biopsy performed just before the patient's death revealed diffuse proliferation of large pleomorphic neoplastic cells with large, round to oval nuclei, vesicular chromatin, and abundant foamy cytoplasm. These cells were positive for histiocytic markers, CD68, lysozyme, CD21, and S-100 protein, but negative for B-cell, T/NK-cell, and epithelial cell markers, thus confirming the presence of histiocytic sarcoma.
Subject(s)
Humans , Male , Middle Aged , Antigens, CD/metabolism , Platelet Endothelial Cell Adhesion Molecule-1/metabolism , Antigens, Differentiation, Myelomonocytic/metabolism , Bone Marrow/pathology , Fever/diagnosis , Histiocytic Sarcoma/diagnosis , Muramidase/metabolism , S100 Proteins/metabolism , Tomography, X-Ray ComputedABSTRACT
The diagnosis of malignant histiocytosis requires a high index of clinical suspicion, awareness of its atypical features and availability of various tissue samples for morphological and special studies. The case reported here highlights the diagnostic difficulties encountered in a patient diagnosed as malignant histiocytosis who presented with cutaneous lesions in multiple foci, which included the face, groin and forearm. Only after repeated biopsies and special stains, a diagnosis of malignant histiocytosis was arrived at. Chemotherapy with CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone) was initiated. The response to chemotherapy was good and the patient is doing well eleven months after initial diagnosis.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Facial Neoplasms/diagnosis , Forearm , Groin , Histiocytic Sarcoma/diagnosis , Humans , Male , Middle Aged , Prednisone/therapeutic use , Skin Neoplasms/diagnosis , Vincristine/therapeutic useABSTRACT
The authors review recent data concerning histiocytosis It is about histiocytosis proliferation that has variable evolution and different prognosis. Three chapters are classified. The reactionals histiocytosis are the most frequent to adults. Some reported demonstrature cases testified it
Subject(s)
Humans , Male , Female , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytic Sarcoma/diagnosis , Histiocytosis, Non-Langerhans-Cell/diagnosis , /classificationABSTRACT
Las histocitosis infantiles son un grupo de enfermedades diversas y poco frecuentes, de etiología desconocida y de difícil diagnóstico clínico. Los estudios anatomopatológicos han permitido su clasificación y enfoque terapéutico. En casi todos los procesos que agrupa la histiocitosis, la boca puede presentar lesiones con una frecuencia de hasta el 70 por ciento, afecta los tejidos duros y blandos y a veces es el odontólogo el primero en observar al paciente. El objetivo de esta comunicación es realizar el diagnóstico diferencial de lesiones de histiocitosis, analizar su evolución con y sin tratamiento y orientar sobre su pronóstico. Sobre 13 niños de 1 a 14 años se siguió la evolución de las lesiones, se realizó el diagnóstico histopatológico y el tratamiento dentro de un equipo multidisciplinario
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Adolescent , Dental Care for Children/methods , Diagnosis, Differential , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Non-Langerhans-Cell/diagnosis , Histiocytic Sarcoma/diagnosis , Histiocytosis/classification , Histiocytosis/diagnosis , Histiocytosis/therapy , Gingival Diseases/etiology , Eosinophilic Granuloma/diagnosis , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis/drug therapy , Histiocytosis/radiotherapy , Mouth Diseases/etiology , Patient Care Team , Periodontal Diseases/etiology , Recurrence/prevention & controlABSTRACT
Se reporta un caso de linfadenitis necrotizante o enfermedad de Kikuchi-Fujimoto. Para nuestro conocimiento es el primer caso en la literatura nacional. Paciente masculino de 14 años de edad, quién consultó por aumento de volúmen de los ganglios linfáticos cervicales, de tres meses de evolución, cuya biopsia fue reportada compatible con histiocitosis maligna. Dadas las buenas condiciones del paciente y la tendencia a la regresión de las adenomegalias se solicita revaloración del material histológico, siendo reportado éste como enfermedad de Kikuchi-Fujimoto. Despúes de dos años de observación, el paciente gozaba de buena salud y ausencia de adenomegalias
Subject(s)
Adolescent , Humans , Male , Histiocytic Sarcoma/diagnosis , Lymphadenitis/diagnosisABSTRACT
An 18-year-old woman had unusual clinical manifestations of malignant histiocytosis (MH) including a long duration of massive splenomegaly, absence of lymphadenopathy, and a number of exacerbations characterized by sudden onset of fever. During life, a definite diagnosis could not be established. The antemortem pathology of the marrow and liver was nonspecific and non-diagnostic. The diagnosis of MH was made after postmortem examination by histologic and immunohistochemical studies. The long duration of massive splenomegaly suggests that this patient could represent a chronic form of MH.
Subject(s)
Adolescent , Female , Histiocytic Sarcoma/diagnosis , Humans , Liver/pathology , Lung/pathology , Spleen/pathologyABSTRACT
Se informa el caso de un joven de 14 anos con hemoglobinopatia SC que tuvo un sindrome hemofagocitico (SH) secundario a salmonelosis. Se discuten las caracteristicas de este sindrome y su diagnostico diferencial. Se hace enfasis en la importancia de considerarlo entre las posibilidades diagnosticas dada la frecuencia en Colombia de las infecciones que pueden desencadenarlo, a saber: tuberculosis, salmonelosis y leishmaniasis
We report a case of hemophagocytlc syndrome occurrlng In a 14 year old boy with SC hemoglobinopathy and salmonellosis; the clinical features of the syndrome are discussed; the need to consider this syndrome in the differential diagnosis is emphazised, since several of the infections that may lead to It- tuberculosis, salmonellosis, lelsmaniasis are frequently found in colombian patients
Subject(s)
Humans , Male , Adolescent , Histiocytic Sarcoma/diagnosis , Anemia, Sickle Cell , Salmonella Infections/complications , Colombia , HemoglobinopathiesABSTRACT
La Reticulosis Medular Histiocítica constituye una neoplasia maligna generalmente fatal en un período corto de tiempo. Los histiocitos atípicos invaden hueso, médula ósea, higado, bazo, intestino, meninges, piel agregando nosotros laringe, localización no referida en la bibliografia consultada. Las lesiones cutáneas constituyen el 10-13% del compromiso general; consisten en tumores queratósicos ulcerados, distribuidos en tronco y miembros. Los métodos inmunohistoquimicos (lisozima, alfa, antitripsina, S-100) son actualmente otro elemento importante de diagnóstico, siendo como en nuestro caso positivos tanto en laringe como piel. La evolución es mala, falleciendo en poco tiempo por sobreinfecciones o bien hemorragias. Nuestra paciente respondió satisfactoriamente a la quimioterapia instituida, en un período de dos años de control.