Disseminated mucocutaneous trichosporonosis in a patient with histiocytic sarcoma

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.

Pediatric multifocal histiocytic sarcoma- a fatal diagnosis not to miss!

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.

Estudio Observacional: Dermatofibrosarcoma Protuberans, Serie de casos / Observational Study: Dermatofibrosarcoma Protuberans, Case Series

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

Bony Histiocytic Sarcoma of the Scapula: Case Report / Sarcoma histiocítico ósseo em escápula: relato de caso

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.

Primary Histiocytic Sarcoma Presenting as a Breast Mass: A Case Report / 한국유방암학회지

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.

Fas-ligand and caspase-3 positivity in three cases of histiocytic sarcoma: a different etiopathogenic pathway?

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.

Bone marrow infiltration of histiocytic sarcoma

No abstract available.

Periarticular histiocytic sarcoma of a thoracic limb in a Rottweiler / 대한수의학회지

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.

Hepatic Involvement of Histiocytic Sarcoma: CT and MRI Findings

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.

A Case of Histiocytic Sarcoma Arising in Head and Neck Region with Rhabdoid Differentiation / 대한이비인후과학회지

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.

Histiocytic Sarcoma of the Cervical Spinal Cord with Secondary Involvement of the Skin / 대한피부과학회지

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.

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face.

Primary Histiocytic Sarcoma of the Central Nervous System / Journal of the Korean Cancer Association, 대한암학회지

Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervous system (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient with primary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord, who had been initially misdiagnosed as demyelinating disease. Two biopsies were necessary before a correct diagnosis was made. A histologic examination showed bizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells were positive for CD68, CD163, and S-100 protein. As a resection was not feasible due to multifocality, he was treated with highdose methotrexate, but showed no response. As a result, he was switched to high dose cytarabine; but again, showed no response. The patient died 2 months from the start of chemotherapy and 8 months from the onset of symptoms. Since few patients with this condition have been described and histopathology is difficult to diagnose, suspicion of the disease is essential.

Primary Gastric Histiocytic Sarcoma Reminiscent of Inflammatory Pseudotumor: A Case Report with Review of the Literature

No abstract available.

Case report of cutaneous histiocytic sarcoma: diagnostic and therapeutic dilemmas / Relato de um caso de sarcoma histiocitico cutaneo: dilemas diagnosticos e terapeuticos

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.

Histomorphological and immunohistochemical characterization of 172 cutaneous round cell tumours in dogs / Caracterização histomorfológica e imuno-histoquímica de 172 neoplasias cutâneas de células redondas em cães

This paper describes the use of a panel of antibodies (CD117, CD3, CD79a, CD45, cytokeratin, vimentin and E-cadherin) on formalin-fixed, paraffin-embedded sections of canine cutaneous round cell tumours. Neoplastic tumours were diagnosed by histology and histochemical stains and included 107 mast cell tumours, 31 cutaneous histiocytomas, two localized histiocytic sarcomas, 21 cutaneous lymphomas, three plasma cell tumours, one transmissible venereal tumour and seven unclassified round cell tumours. The histologic diagnosis was modified in 39.5% of the total 172 neoplasms. The staining for CD45 and Ecadherin were variable, and therefore, the final diagnoses of cutaneous histiocytoma and localized histiocytic sarcoma were made based on histology in association with negative results for CD3, CD79a, CD117 and cytokeratin. The cellular origin of unclassified round cell tumours was defined in all cases. Cutaneous B-cell lymphoma and plasma cell tumours were CD79a-positive and could be distinguished from each other by the morphological characteristics. Mast cell tumours and T cell lymphoma were CD117 and CD3 positive, respectively. The positive staining for vimentin and the negative staining for CD3, CD79a, CD117 and cytokeratin favoured the diagnosis of transmissible venereal tumours. Thus, the final diagnosis of cutaneous round cell tumours should be based on the interpretation of immunohistochemical results together with the cellular morphology observed by histology. Therefore, more studies to optimize the specific markers in formalin-fixed, paraffinembedded tissues (especially for histiocytes) are required for definitive diagnosis of round cell tumours in dogs.

Este trabalho descreve o uso de um painel de anticorpos (CD117, CD3, CD79a, CD45, citoqueratina, vimentina e e-caderina em tecidos formalizados e parafinizados para o diagnóstico de neoplasias de células redondas em cães. Os tumores foram diagnosticados usando-se a histopatologia e a marcação imuno-histoquímica. Foram incluídos 107 mastocitomas, 31 histiocitomas cutâneos, 2 sarcomas histiocíticos localizados, 21 linfomas cutâneos, 3 plasmocitomas, 1 tumor venéreo transmissível e 7 tumores de células redondas não classificados. O diagnóstico histológico foi modificado em 39,5% do total de 172 neoplasias. A marcação do anticorpo CD45 e E-caderina foi variável e, nesse sentido, o diagnóstico final de histiocitoma cutâneo e sarcoma histiocítico localizado foi baseado na histologia em associação com os resultados negativos para CD3, CD79a, CD117 e citoqueratina. A origem celular dos tumores de células redondas não classificados foi definida em todos os casos. Linfoma cutâneo de célula B e plasmocitoma foram positivos para CD79a e foram distinguidos entre si pelas características morfológicas. Marcação positiva para vimentina e negativa para CD3, CD79a, CD117 e citoqueratina favoreceram o diagnóstico dos tumores venereos transmissíveis. Assim, o diagnóstico final dos tumores de células redondas foram baseados na interpretação dos resultados da imuno-histoquímica em conjunto com a avaliação das características morfológicas observadas na histologia. Finalmente, mais estudos em relação à padronização de marcadores específicos para tecidos parafinizados (especialmente para histiócitos) são necessários para o diagnóstico definitivo das neoplasias de células redondas em cães.

Multifocal histiocytic sarcoma of the gastrointestinal tract.

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.

Extranodal Rosai-Dorfman disease of upper respiratory tract: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.</p><p><b>METHODS</b>The clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated.</p><p><b>RESULTS</b>Among the 10 cases studied, there were 3 males and 7 females. The age of patients ranged from 20 to 61 years old (mean 38 years). The lesion arose in the nasal cavity (7 cases), nasopharynx (2 cases) or hard palate to trachea (1 case). Most of the patients presented with nasal obstruction, rhinorrhagia or tumor mass in the nasal/nasopharyngeal regions. CT scan often showed the presence of soft tissue lesion without bone destruction. Histologically, extranodal RDD was characterized by light-staining bands alternating with dark-staining bands. The light-staining bands were formed by aggregates of large round or polygonal histiocytes with emperipoiesis. The dark-staining bands were formed by abundant lymphoplasmacytic infiltrates. Immunohistochemical study showed that the histiocytes strongly expressed S-100 protein and partially expressed CD68. Six patients had no recurrence after surgical resection.</p><p><b>CONCLUSIONS</b>Extranodal RDD of the upper respiratory tract is a rare disorder of histiocytic proliferation, which usually involves the nasal cavity and paranasal sinuses. RDD can easily mimic rhinoscleroma, mainly due to the overlapping morphologic appearance. Immunohistochemical study is helpful in the differential diagnosis.</p>