ABSTRACT
Abstract Trichosporon asahii is the causal agent of trichosporonosis. Patients with immunosuppression or hematological malignancies are at higher risk of infection. Skin and mucosal involvement appear as fast-growing papulonodular lesions and necrotic ulcers. Internal organ dissemination is lethal. Therapeutic success depends on the underlying disease. Here, the authors present the first case of disseminated mucocutaneous trichosporonosis in a patient with a post-mortem diagnosis of histiocytic sarcoma, a rare and aggressive haematolymphoid neoplasm. Regretfully, death occurred despite treatment with liposomal amphotericin B and supportive measures, showcasing the fatality of both diseases.
Subject(s)
Humans , Trichosporon , Histiocytic Sarcoma/drug therapy , Trichosporonosis/diagnosis , Trichosporonosis/drug therapy , Basidiomycota , Antifungal Agents/therapeutic useABSTRACT
Introdução: O tumor fibro-histiocítico plexiforme é uma condição clínica rara, com cerca de 150 casos descritos na literatura. Relato do caso: Paciente, 23 anos, sexo feminino, portadora de lesão nodular em região vulvar sem características de malignidade em exames de ultrassonografia. Realizou acompanhamento clínico, tendo apresentado aumento do tamanho da lesão inicial, sendo optado então por realização de ressecção cirúrgica da lesão. Em estudo histopatológico, evidenciou-se lesão compatível com tumor fibro-histiocítico plexiforme, com margens cirúrgicas livres. No seguimento, a paciente foi encaminhada para avaliação oncológica com a intenção de realizar exames complementares de rastreio e descartar diagnósticos morfologicamente semelhantes. Diante dos exames complementares confirmando o diagnóstico inicial e sem evidência de doença metastática após excisão cirúrgica completa com margens livres, foi iniciado seguimento clínico. Conclusão: Relatar o caso é de extrema importância para divulgar a apresentação clínica, etapas diagnósticas, tratamento proposto e compartilhar informações acerca de evolução clínica apresentada.
Introduction: Plexiform fibrohistiocytic tumor is a rare clinical condition, with about 150 cases described in the literature. Case report: 23-years-old, female patient with nodular lesion in vulva without malignancy characteristics on ultrasound image. She underwent clinical follow-up and after an increase in the size of the lesion, surgical resection was opted. Histopathological findings suggested plexiform fibrohistiocytic tumor, with free surgical margins. In follow up, she was referred to oncological evaluation for screening complementary exams and rule out morphologically similar diagnoses. After the complementary exams confirming the initial diagnosis of plexiform fibrohistiocytic tumor and without evidence of metastatic disease after complete surgical excision with free margins, the patient is in clinical follow-up. Conclusion: Reporting the case is extremely important to disseminate the clinical presentation, diagnostic steps, proposed treatment and share information about the clinical evolution presented.
Introducción: El tumor fibrohistiocítico plexiforme es una condición clínica poco frecuente, con unos 150 casos descritos en la literatura. Reporte del caso: Paciente de 23, sexo feminino, años con lesión nodular en una región vulvar sin características malignas en los exámenes de ultrasonido. Se sometió a un seguimiento clínico, habiendo presentado un aumento en el tamaño de la lesión inicial, y se decidió realizar la resección quirúrgica de la lesión. En un estudio histopatológico, se evidenció una lesión compatible con tumor fibrohistiocítico plexiforme con márgenes quirúrgicos libres. Fue referida para la evaluación oncológica, realizó pruebas complementarias para el cribado y para descartar diagnósticos morfológicamente similares. En vista de las pruebas complementarias que confirman el diagnóstico inicial y sin evidencia de enfermedad metastásica después de una escisión quirúrgica completa con márgenes libres, se inició el seguimiento clínico. Conclusión: Informar del caso es extremadamente importante para difundir la presentación clínica, etapas diagnósticas, tratamiento propuesto y compartir información sobre la evolución clínica presentada.
Subject(s)
Humans , Female , Adult , Young Adult , Vulvar Neoplasms/diagnosis , Histiocytoma, Malignant Fibrous/diagnosis , Vulvar Neoplasms/surgery , Histiocytoma, Malignant Fibrous/surgeryABSTRACT
Objective:To improve awareness of the diagnosis and treatment of splenic histiocytic sarcoma.Methods:The clinical data of one patient with splenic histiocytic sarcoma who was admitted to First Teaching Hospital of Tianjin University of Traditional Chinese Medicine in July 2020 were retrospectively analyzed, and the relevant domestic and foreign literature was reviewed.Results:The patient was diagnosed as splenic histiocytic sarcoma by histopathological and immunohistochemical examinations. The methylprednisolone combined with cyclosporine was ineffective. The patient received a total splenectomy, followed by chemotherapy with VECD and CHOP regimens. The patient's condition was stable during the 5-month follow-up after the operation. The result of bone puncture showed that there was no infiltration of histocytic sarcoma, and hematological remission was obtained.Conclusions:Splenic histocytic sarcoma is a highly malignant tumor with insidious onset, unclear pathogenesis, and lack of specificity in clinical manifestations and imaging examinations. The diagnosis depends on pathological biopsy and immunohistochemistry, and needs to be differentiated from other malignant tumors of lymphoid hematopoietic tissue. At present, there is no best treatment for splenic histiocytic sarcoma, and most patients have a poor prognosis.
ABSTRACT
Histiocytic sarcoma (HS) is an extremely rare and aggressive hematopoietic tumor. Although it can be seen at any anatomic location, the most common primary sites are skin as extranodal region, locations including the lymph nodes and gastrointestinal tract. To the best of our knowledge, in light of PubMed search, this is the first primary tonsillar HS case presented with disseminated metastases at the time of diagnosis. A 58-year-old male patient applied with swelling on the right side of the neck, difficulty in swallowing, and weight loss. Positron emission tomography computed tomography was performed and increased pathological 18F fluorodeoxy D glucose uptake was detected in the right palatine tonsil, bilateral cervical multiple lymph nodes, liver masses, intra abdominal lymph nodes, and nodular lesion in the left adrenal gland. Tonsillectomy was performed and the pathological result was reported as HS. The patient did not respond to any treatment and had died after 5 months from the date of diagnosis. In conclusion, HS is generally diagnosed at advanced stage, it has limited chemotherapy response and high mortality rates. To understand this rare disease's pathophysiological and clinical features, further investigations are needed
ABSTRACT
Histiocytic sarcoma (HS) is a rare hematolymphoid malignant neoplasm with an aggressive clinical course. It can arise de novo or from low-grade B-cell lymphoma. We describe the case of a 16-year-old boy referred to our hospital with generalized lymphadenopathy, weight loss, and decreased appetite for one month. The patient died undiagnosed on the 7th day of hospitalization. Lymph node and bone marrow biopsies were performed one day before the patient died. The lymph node biopsy revealed an architectural effacement with a diffuse proliferation of large pleomorphic neoplastic cells containing large, multilobulated nuclei, coarse vesicular chromatin, prominent nucleoli, and a moderate amount of eosinophilic cytoplasm. The bone marrow aspiration smears and biopsy also showed evidence of infiltration by these above-mentioned cells. Based on the morphology, along with the exclusion of many differential diagnoses by an extensive panel of immunohistochemical markers, a diagnosis of HS was made. This case report aims at evaluating all the clinical and immunophenotypic features of a case of HS with multifocal presentation and an aggressive clinical course in order to give a correct and definite diagnosis at the proper time.
Subject(s)
Humans , Male , Adolescent , Histiocytic Sarcoma/pathology , Autopsy , Immunophenotyping , Lymphoma, B-Cell , Fatal Outcome , Diagnosis, Differential , LymphadenopathyABSTRACT
Introducción: El dermatofibrosarcoma protuberans(DFSP), es uno de los tumores cutáneos menos frecuentes, se clasifica dentro de los sarcomas fibrohistociticos y está caracterizado por un crecimiento lento y progresivo. Métodos: Se realizó una serie de casos retrospectiva de los pacientes con DFSP atendidos en el Centro de la Piel (CEPI) entre 2010 y 2019, se tomó en cuenta la información demográfica, características clínicas, tratamiento y su evolución. Resultados: Seis pacientes fueron diagnosticados y tratados por DFSP en el período evaluado, existió un predominio de mujeres (4 mujeres, 2 hombres) con una media de edad de 58.2 (DE =19,6), tres de ellos presentaron lesiones en forma de placa indurada y los restantes presentaron nódulo único, la localización de las lesiones fueron en hombro, cuello e hipogastrio. El tratamiento más utilizado fue la escisión amplia, ninguno de los pacientes presentó recidivas al primer y quinto año del seguimiento. Conclusión: Es necesario el reconocimiento de las características clínicas típicas de DFSP ya que podría pasar como una tumoración benigna de piel, por lo cual se debe apoyar con el estudio histopatológico frente a la sospecha de este tipo de lesión
Introduction: Dermatofibrosarcoma protuberans (DFSP) is one of the rare tumors found in the fibrohistocytic sarcomas and it is characterized by slow and progressive growth. Methods:A retrospective study of six cases treated at the Skin Center (CEPI) between 2010 and 2019 was carried out. We have take into account demographic information, characteristics, treatment and evolution of the lesions. Results:Six patients were diagnosed and treated by DFSP. There was a predominance of women (4 women, 2 men) with a mean age of 58.2 (SD = 19.6), three of them presented lesions in the form of indurated plaque and the rest presented a unique nodule. The locations of the lesions were the shoulder, neck and hypogastrium. The most commonly used treatment was wide excision, none of the patients presented recurrences at the first and fifth year of follow-up. Conclusion:Recognition of the typical clinical characteristics of DFSP is necessary since it could be mistaken as a benign tumor of the skin. The diagnosis must be always done by histopathological confirmation
Subject(s)
Skin , Mohs Surgery , Dermatofibrosarcoma , Histiocytic SarcomaABSTRACT
Histiocytic sarcoma is very rare malignant neoplasm showing morphological and immunophenotypic characteristics of histiocyte. Morphologic diagnosis is challenging due to overlapping histologic features with diverse mimics. Histologic diagnosis is confirmed by expression of one or more histiocytic markers, including CD163, CD68, and lysozyme. We present a bona fida case of extranodal histiocytic sarcoma involving the small intestine in a 67-year-old male. The diagnosis is confirmed by diffuse immunoreactivity of CD163, which is believed to be more specific histiocytic marker than CD68.
ABSTRACT
Histiocytic sarcoma is a rare malignant neoplasm that demonstrates mature histiocytic traits as characterized by immunohistochemistry. We report a case of extranodal histiocytic sarcoma (ENHS) of colon in a 56-year-old man presenting with gastrointestinal symptoms. Radiological findings were indicative of lymphoma or diffuse metastatic disease in colon. Histopathology of colectomy specimen was suggestive of ENHS, and immunohistochemical studies confirmed the uncommon diagnosis. The patient refused further therapy and succumbed to systemic complications of metastatic disease within a month of diagnosis. There have only been seven previous reports in world literature of ENHS involving large intestine.
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Abstract Case report of a pediatric patient with a diagnosis of bony histiocytic sarcoma of the scapula, submitted to oncologic resection (Tikhoff-Linberg type II surgery), who returned to previous activities and was disease-free after 24 months of follow-up.
Resumo Relato de caso de paciente pediátrico com diagnóstico de sarcoma histiocítico ósseo em escápula, submetido à ressecção oncológica (cirurgia de Tikhoff-Linberg tipo II), com retorno às atividades prévias e livre de doença após 24 meses de seguimento.
Subject(s)
Humans , Male , Adolescent , Histiocytic Sarcoma/surgery , Histiocytic Sarcoma/pathology , Histiocytic Sarcoma/diagnostic imagingABSTRACT
Objective@#To study the clinicopathological features, differential diagnosis and prognosis of primary histiocytic sarcoma of central nervous system(CNS).@*Methods@#Three cases of CNS histiocytic sarcoma were collected at Chinese People′s Liberation Army General Hospital from 2005 to 2018. Their clinicopathological characteristics were analyzed, and the related literature reviewed.@*Results@#The three patients included two females and one male, aged 36, 44, 58 years (median 44 years). MRI showed heterogeneously enhancing lesions which were considered meningioma, high-grade glioma or metastatic carcinoma. Histopathologically there were moderately pleomorphic, mitotically active tumor cells with a loose arrangement, effacing the normal brain tissue. These cells possess abundant eosinophilic cytoplasm, highly atypical nuclei, predominant nucleoli, and hemophagocytosis; multinucleated or spindled forms were also seen, as was background reactive inflammation. The tumor cells were typically positive for CD68, CD163, vimentin and lysozyme, S-100 protein, two of three cases were positive for BRAF V600E,one of three cases was partly positive for CD45, CD45RO, CD4, CD34, and negative for GFAP, Olig-2, CK, EMA, SSTR2, CD99, CD117, MPO, CD1a, Langerin, CD21, CD23, CD35, CD15, CD30, CD38, and CD138. The index of Ki-67 was 30%-75%. Rich reticular fiber network was seen in all cases; BRAF V600E mutation was present in two cases.@*Conclusions@#CNS histiocytic sarcoma is a rare malignant tumor; histopathologic and immunohistochemical examination are necessary for the diagnosis and to exclude other primary CNS and hematolymphopoietic tumors. Primary CNS histiocytic sarcoma is treated by surgery, chemotherapy and radiation therapy, but the prognosis is poor. Complete resection combined with high dose focused radiotherapy can improve the prognosis.
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Histiocytic sarcoma is a rare hematologic malignancy, with very few cases of primary histiocytic sarcoma of the breast described in English scientific literature. Herein, we describe a case of primary histiocytic sarcoma of the breast in a 75-year-old woman, with no clinical history of malignant tumors, who presented with a palpable solitary breast mass. Microscopically, the resected breast mass showed large pleomorphic cells, some multinucleated giant cells, and admixed inflammatory components. The pleomorphic tumor cells further showed a diffuse, noncohesive growth pattern, an abundant eosinophilic cytoplasm, and strong and diffuse immunoreactivity for cluster of differentiation (CD) 68 and CD163. Furthermore, a whole-body positron-emission tomography/computed tomography using deoxy-2-[¹⁸F]fluoro-D-glucose performed after surgery showed no other masses or lesions. After surgical excision, the patient was followed up, and no evidence of tumor recurrence or metastasis was noted.
Subject(s)
Aged , Female , Humans , Breast , Cytoplasm , Eosinophils , Giant Cells , Hematologic Neoplasms , Histiocytes , Histiocytic Sarcoma , Neoplasm Metastasis , RecurrenceABSTRACT
Histiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunophenotypic evidence of histiocytic differentiation. We describe a rare case of synchronous HS in a patient of B-cell acute lymphoblastic leukemia (ALL). A 16-year-old boy diagnosed as ALL also presented with a swelling over the right Achilles tendon. The cytological features of the swelling suggested a histiocytic lesion. Histological and immunohistochemical examination clinched the diagnosis of HS. The available 5-year follow-up showed no recurrence. It was a diagnostic dilemma on fine-needle aspiration. We discuss the cytological features of HS which can help in reaching a diagnosis and emphasize that it should be considered in the differential diagnosis for unexplained swellings in patients of hematological malignancies. Wide local excision of localized HS is associated with a long-term favorable outcome.
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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
An 8-year-old, castrated, male Rottweiler was referred for evaluation of chronic right thoracic limb lameness and a progressively growing mass surrounding the right elbow joint. On admission, the dog's general health was good, without abnormalities detected on physical examination. The dog was diagnosed with periarticular histiocytic sarcoma. Although draining lymph nodes and lung metastases were suspected, palliative amputation was performed. Localized histiocytic sarcomas, with destructive lesions involving multiple bones of a joint and periarticular soft-tissue masses, are uncommon in dogs. This case report presents clinical findings, imaging characteristics, and histopathologic and immunohistochemical features of a periarticular joint histiocytic sarcoma.
Subject(s)
Animals , Child , Dogs , Humans , Male , Amputation, Surgical , Elbow Joint , Extremities , Histiocytic Sarcoma , Immunohistochemistry , Joints , Lung , Lymph Nodes , Neoplasm Metastasis , Physical ExaminationABSTRACT
El sarcoma histiocítico es un desorden de células no Langerhans extremadamente raro con un curso clínico agresivo y limitadas opciones de tratamiento. Se presenta el caso de un paciente adulto joven de género masculino, con cuadro febril prolongado, compromiso hematológico persistente, hepatoesplenomegalia y linfadenopatias generalizadas con curso clínico fatal, en quien por las características clínicas, paraclínicas y de estudios histopatológicos como de inmunohistoquímica se llegó a dicha presunción diagnostica
Histiocytic sarcoma is an extremely rare non-Langerhans cell disorder with an aggressive clinical course and limited options for therapy. We present the case of a young adult male patient, with prolonged fever, persistent hematological involvement, hepatosplenomegaly, and generalized lymphadenopathy with a fatal clinical course, in whom we arrived to this presumptive diagnosis considering the clinical, paraclinical, and histopathological characteristics, as well as immunohistochemistry
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Objective To investigate the diagnostic criteria, clinical management and prognostic factors of primary central nervous system (CNS) histiocytic sarcoma (HS). Methods An adult patients with primary CNS HS was reported, and literature on the rare entity were reviewed. Results The patient had no previous history of disease. Magnetic resonance imaging (including magnetic resonance spectroscopy) suggested a mass in the frontal lobe with obvious vasogenic edema, which was considered glioma. Surgery was the initial treatment and the tumor was totally removed. The histological findings revealed the HS. The patient received concomitant chemoradiotherapy postoperatively and currently lived without recurrence. Conclusions Primary CNS HS is extremely rare hematopoietic malignant tumor. The pathological feature and comprehensive immunophenotype panel are the useful ways to establish the diagnosis till now. Primary CNS HS should be treated intensively with surgery, chemotherapy, and/or radiation therapy. However, prognosis is disappointed in most of patients. Concomitant chemoradiotherapy might be an alternative treatment.
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Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.
Subject(s)
Aged , Female , Humans , Biopsy , Hematologic Neoplasms , Hepatic Veins , Histiocytic Sarcoma , Iron , Liver , Magnetic Resonance ImagingABSTRACT
Histiocytic sarcoma is a rare kind of histiocytic lymphoma with an undetectable onset, no specific clinical and imaging mani-festations, and rapid progression. At diagnosis, the histiocytic sarcoma has already spread significantly in most patients. The diagnosis of histiocytic sarcoma depends on the basis of its mitotic figures and at least expression of one of the following:CD68, CD163, and lyso-zyme. Operation excision, radiotherapy, and chemotherapy are currently the more common treatment methods for histiocytic sarco-ma. However, no unified curative and treatment standard exists, and prognosis is poor. This review summarizes the diagnosis and treat-ment of histiocytic sarcoma.
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Head and neck sarcomas account for about 1% of malignant head and neck tumors, and histiocytic sarcomas (HSs) account for less than 1% of all hematolymphoid neoplasms. These rare hematopoietic neoplasms are the result of a malignant proliferation of cells. They have morphological and immunophenotypic features of mature tissue histiocytes, which play a major role in processing and presenting antigens to T or B lymphocytes. The mesenchymal differentiation of HSs has not been reported. We describe a case of HS with rhabdoid differentiation in a 55-year-old man who presented with a 3×2.5×2 cm mass in the left supraclavicular area. To the best of our knowledge, this is the first report worldwide of a primary HS with rhabdoid differentiation.
Subject(s)
Humans , Middle Aged , B-Lymphocytes , Head , Hematologic Neoplasms , Histiocytes , Histiocytic Sarcoma , Neck , SarcomaABSTRACT
Histiocytic sarcoma is a rare, lymphohematopoietic malignant tumor comprised of tumor cells with the morphological and immunophenotypic features of mature histiocytes. A 35-year-old man presented with a disseminated histiocytic sarcoma that first occurred in the spinal cord and metastasized to the skin and lymph nodes. The tumor cells of the primary histiocytic sarcoma of the spinal cord were very large epithelioid cells with abundant eosinophilic cytoplasms and large, round-to-oval nuclei. In contrast, the metastatic histiocytic sarcoma of the skin was composed of relatively small polygonal cells with well-defined cell borders and high N/C (nucleus/cytoplasm) ratios. Immunohistochemically, both tumors were diffusely positive for histiocyte-associated antigens; but negative for epithelial, melanocyte, lymphoid, and dendritic cell antigens. It is important to recognize the morphological features and immunohistochemical characteristics of metastatic cells in order to ensure accurate diagnoses.